An intramedullary space-occupying lesion in the form of an epidermoid cyst was diagnosed in a one-and-a-half-year-old flat-coated retriever. Dorsal laminectomy and durotomy were performed in order to establish the diagnosis followed by excision of one third of the cyst. The remaining cystic tissue that was intimately attached to the spinal cord parenchyma was left in place in order to avoid further damage to the nervous tissue. The dog's neurological status improved dramatically after the surgery, but deteriorated four months later due to recurrence of the cyst.