A 2-year-old female Rottweiler was examined because of progressive gait deficits consistent with transverse myelopathy between the T2 and L3 spinal cord segments. Myelography confirmed an intramedullary spinal cord lesion between T13 and L1, so the dog was euthanatized. At necropsy, an intramedullary epidermoid cyst was confirmed in the T13 and L1 spinal cord segments. The epidermoid cyst probably resulted from a congenital cause.