Darier Disease is an inherited skin disorder affecting dogs. Affected dogs typically present with skin lesions prior to six months of age. These lesions may include multiple crusted plaques within the ears and/or overlying skin nodules disseminated around the body. These lesions override cysts filled with abnormal skin cells. Treatment includes veterinary assessment of the presenting lesions, managing secondary infections and therapeutic excision of the cysts. Skin biopsies should be taken to assist in diagnosis and distinguish the presentation of Darier Disease from other dermatologic disorders. Dogs with Darier Disease should be monitored for recurrence as lesions may develop over several years.