MAJOR SHAREHOLDER ANNOUNCEMENT

Copenhagen, Denmark, October 2, 2019 – Orphazyme A/S (ticker: ORPHA.CO), a biopharmaceutical company dedicated to developing treatments for patients living with rare diseases, hereby announces the receipt of notification pursuant to Section 38 of the Danish Capital Markets Act from Danske Bank A/S that Danske Bank as of October 1, 2019 holds a total 998,962 shares in the Company, corresponding to 4.99% of the share capital and that Danske Bank as of October 1, 2019 controls 7.74% of the voting rights in the Company.

Danske Bank A/S’ shareholding consists of a 4.85% indirect and 0.14% direct ownership through Danica Pension Livsforsikringsaktieselskab, Danica Pension Försäkringsaktiebolag, Investeringsforeningen Danske Invest and Danske Invest SICAV. Danske Bank A/S’ control of voting rights in the Company consists of a 7.59% indirect and 0.14% direct control through Danica Pension Livsforsikringsaktieselskab, Danica Pension Försäkringsaktiebolag, Investeringsforeningen Danske Invest, and Danske Invest SICAV.