The authors report, after a follow-up period of two years, a case of intramedullary congenital epidermoid cyst treated surgically. The patient, a 21 year-old man, presented with a seven years history of motor disturbances of his left lower limb and more recent urinary incontinence. The diagnosis of an intramedullary tumor was provided by a myelogram and a post-myelogram CT. In the absence of a dermal sinus or vertebral dysraphic anomalies, the epidermoid nature of the mass was an operative and histological finding. The main data from the literature are commented: frequency, topography, clinical diagnosis, and radiologic or M.R.I. imaging. Intracapsular removal of the mass seems to result, as in the presently reported case, in a satisfactory outcome.