MAJOR SHAREHOLDER ANNOUNCEMENT

Copenhagen, December 18, 2017 – With reference to company announcement no. 5/2017 dated November 16, 2017 regarding the result of the Initial Public Offering (the “Offering”) of Orphazyme A/S (“Orphazyme”) and company announcement no. 10/2017 dated November 16, 2017, Orphazyme hereby announces that it has received the following notification pursuant to section 29 of the Danish Securities Trading Act regarding Novo Holdings A/S’ holding of shares in Orphazyme.

Novo Holdings A/S has increased its direct shareholding in Orphazyme as a consequence of having been redelivered 1,125,000 existing shares in Orphazyme of a nominal value of DKK 1 each previously lent to Carnegie Investment Bank, filial af Carnegie Investment Bank AB (publ), Sverige and Danske Bank A/S (the “Joint Global Coordinators”) for the purposes of delivery of shares to investors as a result of the overallotment option in connection with Orphazyme’s initial public offering of shares.

Accordingly, Novo Holdings A/S’ holding of 3,830,832 shares of a nominal value of DKK 1 each in Orphazyme will correspond to 19.2% of the total share capital and voting rights.

Novo Holdings A/S is a limited liability company organized under the laws of Denmark under CVR no. 24257630 with its registered office at Tuborg Havnevej 19, 2900 Hellerup, Denmark. Novo Holdings A/S is wholly-owned by the Novo Nordisk Foundation.

The Novo Nordisk Foundation is a foundation organised under the laws of Denmark under CVR no. 10582989 with its registered office at Tuborg Havnevej 19, DK-2900 Hellerup, Denmark.