Epidermoid cysts are classical tumors in neurosurgery, but spinal locations are rare and intramedullary locations represent only approximately 80 cases in the literature. Intramedullary epidermoid cysts arise from ectodermic tissue inclusion during neural tube closure. Intramedullary epidermoid cysts are more frequent in young patients and in a thoracic location. We report the case of a 61-year-old patient consulting for bilateral asymmetrical progressive paraparesis and proprioceptive ataxia revealing a T3–T4 exophytic medullar epidermoid cyst. The tumor was surgically removed with a favorable outcome. The capsule was partially removed. The authors present an updated literature review of intramedullary epidermoid cyst diagnosis, management and recommendations.