Exhibit 99.1

FOR IMMEDIATE RELEASE

BioMarin Announces Fourth Quarter and Full Year 2016 Financial Results

- Full Year 2016 Total BioMarin Revenues Increase 26% Year over Year to $1.12 billion

- Full Year 2017 Total BioMarin Revenue Guidance of Between $1.25 billion to $1.30 billion

Financial Highlights (in millions of U.S. dollars, except per share data, unaudited)

SAN RAFAEL, Calif., February 23, 2017 – BioMarin Pharmaceutical Inc. (NASDAQ: BMRN) today announced financial results for the fourth quarter and year ended December 31, 2016. For the quarter ended December 31, 2016, GAAP net loss was $91 million, or ($0.53) per basic and diluted share, compared to GAAP net income of $69 million, or $0.43 and $0.39 per basic and diluted share, respectively, for the fourth quarter of 2015. GAAP net loss for the year ended December 31, 2016 was $630 million, or ($3.80) and ($3.81) per basic and diluted share, respectively, compared to GAAP net loss of $172 million, or ($1.07) per basic and diluted share for the year ended December 31, 2015. The

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change in GAAP net loss year over year was primarily due to the impairment of intangible assets associated with the discontinuance of the Kyndrisa and reveglucosidase alfa programs in 2016 and the absence of the gain on the sale of rights to talazoparib in 2015.

Non-GAAP loss for the year ended December 31, 2016 was $36 million, compared to non-GAAP loss of $143 million for the year ended December 31, 2015. Non-GAAP income (loss) is defined by the Company as reported GAAP net income (loss), excluding net interest expense, provision for (benefit from ) income taxes, depreciation expense, amortization expense, stock-based compensation expense, contingent consideration expense and certain other specified items as detailed below. Refer to Non-GAAP Information beginning on page 9 of this press release for a complete discussion of the Company’s non-GAAP financial information and reconciliations to the comparable GAAP reported information.

Total BioMarin Revenues were $1.12 billion for the year ended December 31, 2016, an increase of 26% compared to the same period in 2015. For the fourth quarter of 2016, Total BioMarin Revenues were $300 million, an increase of 32% compared to the same period in 2015. Vimizim net product revenues increased to $94 million in the quarter, a 59% year over year increase, primarily driven by 40% growth in new patients on Vimizim therapy year over year. For the fourth quarter of 2016, Naglazyme net product revenues increased to $75 million, an increase of 25% year over year. Patients on Naglazyme therapy continue to show consistent growth with an increase of 9% year over year.  For the fourth quarter of 2016, Kuvan net product revenues increased to $90 million, a 38% year over year increase, including $70 million contributed from revenue in North America. Growth was driven by a 15% increase in patients on Kuvan therapy in North America, and $20 million contributed from net product revenues in ex-North American territories newly acquired in 2016. 

As of December 31, 2016, BioMarin had cash, cash equivalents and investments totaling $1.4 billion, which includes $713 million of net proceeds from the August 12, 2016 public offering, as compared to $1.0 billion on December 31, 2015.

Commenting on the year, Jean-Jacques Bienaimé, Chairman and Chief Executive Officer of BioMarin said, “2016 was a transformational year for BioMarin. Our commercial business topped $1 billion for the first time while we concurrently achieved significant clinical and regulatory milestones across the product pipeline. In the third quarter, we shared proof-of-concept data with BMN 270 gene therapy, the only factor VIII product in clinical development for the treatment of severe Hemophilia A. In addition, our regulatory filings for approval of Brineura, for the treatment of Batten disease, were accepted and validated in both the U.S. and EU. With the Prescription Drug User Fee Act (PDUFA) goal date for an FDA approval decision of April 27, 2017, we hope to have an approved treatment option for this devastating childhood disease in the near future.”

Mr. Bienaimé continued, “In addition, in 2016 we moved our vosoritide program forward based on Phase 2 results in children ages 5-14 with achondroplasia. At the end of 2016, we initiated a one-year, randomized, placebo-controlled Phase 3 study in children with achondroplasia ages 5-14 using a daily 15µg/kg dose. We recently provided encouraging preliminary results with our earliest clinical-stage program BMN 250 for the treatment of MPS IIIB, or Sanfilippo Syndrome, Type B. We are now moving to the expansion phase of the development program that will assess the impact of treatment with BMN 250 on the neurocognitive function in this rapidly progressive pediatric brain disease. Finally, in addition to the filing of the BLA for pegvaliase for the treatment of phenylketonuria expected in the second quarter of 2017, we expect to start to turn the corner to profitability with the achievement of positive non-GAAP results for the full year 2017.”  

Revenues (in millions of U.S. dollars, unaudited)

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Total BioMarin Revenues

Details of Net Product Revenues Attributable to Aldurazyme

2017 Financial Guidance

Revenue Guidance ($ in millions)

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Select Income Statement Guidance ($ in millions, except percentages)

Key Program Updates

On February 1, 2017, BioMarin Pharmaceutical Inc. announced that the European Medicines Agency (EMA) had granted access to its Priority Medicines (PRIME) regulatory initiative for the company's investigational gene therapy treatment for severe hemophilia A, BMN 270.

To be accepted for PRIME, an investigational therapy has to show its potential to benefit patients with unmet medical needs based on early clinical data. BMN 270 gene therapy is the first and only product candidate for hemophilia A to have received this designation.

On January 9, 2017, the Company announced an update to its positive interim results of an open-label Phase 1/2 study with BMN 270. These data were an update from previously reported results in July 2016. A total of nine patients with severe hemophilia A received a single dose of BMN 270, seven of whom have been treated at the highest dose of 6 x 10¹³ vg/kg.

As of the December 9, 2016 data cutoff, post-treatment follow-up ranges from 34 to 50 weeks.  Median Factor VIII levels for the high dose cohort have been consistently within the normal range from 20 weeks through 44 weeks of treatment. According to the World Federation of Hemophilia rankings of severity of hemophilia A, the normal range of Factor VIII activity levels is between 50%-150%, expressed as a percentage of normal factor activity in blood.  For those seven patients, as of each patient's most recent reading, six of seven patients continue to have Factor VIII activity levels above 50% and the seventh continues to be above 15%, which is the mild range (5%-40%) of hemophilia A.  

For the six patients at the high dose and previously on a Factor VIII prophylactic regimen, the mean annualized bleeding rate dropped 91% from 16.3 before the BMN 270 infusion to 1.5 two weeks after being dosed (median annualized bleeding rate dropped from 16.5 to 0). For those same six patients, the mean annualized Factor VIII infusions fell 98% from 136.7 to 2.9 (median annualized Factor VIII infusions fell from 138.5 to 0).

In addition,  six of the seven patients at the high dose as of January 9, 2017 were within the normal alanine aminotransferase (ALT) range, and one patient was less than 5% above the upper limit of normal, which is 43 U/L for the central laboratory in this study. At that time all patients had successfully tapered off of steroids with no lasting significant impact on Factor VIII expression or ALT levels.

The Company intends to provide the next program update by the start of the potentially registration enabling Phase 2b study in 3Q 2017.  In addition, the company is expected to commission its commercial gene therapy manufacturing facility by mid-2017.

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The Phase 3 study is a randomized, placebo-controlled study of vosoritide in approximately 110 children with achondroplasia ages 5-14 for 52 weeks. The study will be followed by a subsequent open-label extension. Children in this study will have completed a minimum six-month baseline study to determine their respective baseline growth velocity prior to entering the Phase 3 study. Vosoritide is being tested in children whose growth plates are still open. This is approximately 25 percent of people with achondroplasia.

The primary endpoint of the study is the change in growth velocity from baseline over one year in children treated compared to placebo. The company also plans to augment the growth velocity data in the Phase 3 study with assessments of proportionality, functionality and cumulative growth observed in that study and the ongoing Phase 2 study.

The FDA granted Brineura Priority Review status, which is designated for drugs that offer major advances in treatment or provide a treatment where no adequate therapy exists. Brineura was previously granted Orphan Drug Designation and Breakthrough Therapy Designation by the FDA. BioMarin also received validation of the Marketing Authorization Application (MAA) to the EMA for Brineura. Assuming a positive opinion from the Committee for Medicinal Products for Human Use (CHMP) and standard assessment timing, a decision from the European Commission is anticipated by the third quarter of 2017. The EMA previously granted Brineura Orphan Drug Designation.

Conference Call Details

BioMarin will host a conference call and webcast to discuss fourth quarter and full year 2016 financial results today, Thursday, February 23, 2017 at 4:30 p.m. ET. This event can be accessed on the investor section of the BioMarin website at www.BMRN.com.

U.S. / Canada Dial-in Number: 866.502.9859

International Dial-in Number: 547.990.1362

Conference ID: 60631310

Replay Dial-in Number: 855.859.2056

Replay International Dial-in Number: 404.537.3406

Conference ID: 60631310

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About BioMarin

BioMarin is a global biotechnology company that develops and commercializes innovative therapies for patients with serious and life-threatening rare and ultra-rare genetic diseases. The Company's portfolio consists of five commercialized products and multiple clinical and pre-clinical product candidates. For additional information, please visit www.BMRN.com.

Forward-Looking Statement

This press release contains forward-looking statements about the business prospects of BioMarin Pharmaceutical Inc. (BioMarin),  including, without limitation, statements about: the expectations of total BioMarin revenues, net product revenues and expenses for BioMarin’s commercial products, GAAP net loss, non-GAAP income (loss) and other specified income statement guidance; the potential revenues and expenses related to BioMarin’s product candidates, including BMN 270, Brineura, pegvaliase, vosoritide and BMN 250; the financial performance of BioMarin as a whole; the timing of BioMarin's clinical studies and trials and announcements of data from those studies and trials; the continued clinical development and commercialization of BioMarin’s commercial products and product candidates; the possible approval and commercialization of BioMarin’s product candidates; and actions by regulatory authorities. These forward-looking statements are predictions and involve risks and uncertainties such that actual results may differ materially from these statements. These risks and uncertainties include, among others: BioMarin’s  success in the commercialization of its commercial products; Genzyme Corporation's success in continuing the commercialization of Aldurazyme; results and timing of current and planned preclinical studies and clinical trials, BioMarin’s  ability to successfully manufacture its commercial products and product candidates; the content and timing of decisions by the FDA, the European Commission and other regulatory authorities concerning each of the described products and product candidates; the market for each of these products; actual sales of BioMarin’s commercial products; and those factors detailed in BioMarin's filings with the Securities and Exchange Commission (SEC), including, without limitation, the factors contained under the caption "Risk Factors" in BioMarin's Quarterly Report on Form 10-Q for the quarter ended September 30, 2016 as such factors may be updated by any subsequent reports. Stockholders are urged not to place undue reliance on forward-looking statements, which speak only as of the date hereof. BioMarin is under no obligation, and expressly disclaims any obligation to update or alter any forward-looking statement, whether as a result of new information, future events or otherwise.

BioMarin^®, Vimizim^®, Naglazyme^®, Kuvan^® and Firdapse^®  are registered trademarks of BioMarin Pharmaceutical Inc., or its affiliates.  Brineura^TM and Kyndrisa^TM are trademarks of BioMarin Pharmaceutical Inc. Aldurazyme^® is a registered trademark of BioMarin/Genzyme LLC.

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BIOMARIN PHARMACEUTICAL INC.

CONDENSED CONSOLIDATED BALANCE SHEETS

December 31, 2016 and December 31, 2015

(In thousands of U.S. dollars, except share and per share amounts)

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BIOMARIN PHARMACEUTICAL INC.

CONDENSED CONSOLIDATED STATEMENTS OF OPERATIONS

Three and Twelve Months Ended December 31, 2016 and 2015

(In thousands of U.S. dollars, except per share amounts)

(Unaudited)

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Non-GAAP Information

The results presented in this press release for the three and twelve months ended December 31, 2016 and 2015 as well as 2017 full-year guidance include both GAAP information and non-GAAP information. As used in this release, non-GAAP income (loss) is defined by the Company as GAAP net loss excluding net interest expense, provision for (benefit from) income taxes, depreciation expense, amortization expense, stock-based compensation expense, contingent consideration expense and certain other specified items, as detailed below. In addition, BioMarin includes in this press release the effects of these adjustments on certain components of GAAP net loss for each of the periods presented. In this regard, non-GAAP income (loss) and its components, including non-GAAP Cost of sales, non-GAAP Research and development expenses, non-GAAP Selling, general and administrative expense, non-GAAP Intangible asset amortization and contingent consideration, non-GAAP Other income (expense) and non-GAAP Provision for (benefit from) income taxes are statement of operations line items prepared on the same basis as, and therefore components of, the overall non-GAAP measures.

BioMarin regularly uses both GAAP and non-GAAP results and expectations internally to assess its financial operating performance and evaluate key business decisions related to its principal business activities – the discovery, development, manufacture, marketing and sale of innovative biologic therapies. Because non-GAAP income (loss) and its components are important internal measurements for BioMarin, the Company believes that providing this information in conjunction with BioMarin’s GAAP information enhances investors’ and analysts’ ability to meaningfully compare the Company’s results from period to period and to its forward looking guidance, and to identify operating trends in the Company’s principal business.

Non-GAAP income (loss) and its components are not meant to be considered in isolation, as a substitute for, or superior to comparable GAAP measures and should be read in conjunction with the consolidated financial information prepared in accordance with GAAP. Investors should note that the non-GAAP information is not prepared under any comprehensive set of accounting rules or principles and does not reflect all of the amounts associated with the Company’s results of operations as determined in accordance with GAAP. Investors should also note that these non-GAAP measures have no standardized meaning prescribed by GAAP and, therefore, have limits in their usefulness to investors. In addition, from time to time in the future there may be other items that the Company may exclude for purposes of its non-GAAP measures; likewise, the Company may in the future cease to exclude items that it has historically excluded for purposes of its non-GAAP measures. Because of the non-standardized definitions, the non-GAAP measure as used by BioMarin in this press release and the accompanying tables may be calculated differently from, and therefore may not be directly comparable to, similarly titled measures used by other companies.

The following table presents the reconciliation of GAAP Net Income (Loss) to non-GAAP Income (Loss):

Reconciliation of GAAP Net Income (Loss) to non-GAAP Income (Loss)

(In millions of U.S. dollars)

(unaudited)

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The following reconciliation of the GAAP reported to non-GAAP information provides the details of the effects of the non-GAAP adjustments on certain components of the Company’s operating results for each of the periods presented.

   Reconciliation Of Certain GAAP Reported Information To non-GAAP Information

Three and Twelve Months Ended December 31, 2016 and 2015

(In millions of U.S. dollars)

(Unaudited)

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