A 2-year-old, male, Burmese cat was evaluated for chronic progressive hindlimb weakness, ataxia and urinary incontinence. Radiographic examination, myelography and magnetic resonance imaging defined congenital vertebral anomalies and a space-occupying intradural, extramedullary mass. A dermoid sinus was subsequently identified dorsal to the affected spine. Surgical excision of the tract necessitated a dorsal laminectomy and removal of a 1-cm diameter intradural dermoid sinus ‘cyst’ that contained hair and sebaceous debris. The cat recovered hindlimb function after surgery and remains asymptomatic 50 months after surgery except for a persistent inability to urinate voluntarily.