Two cases of diastematomyelia are presented and the related literature is reviewed. Diastematomyelia, a complete or incomplete sagittal division of the neural axis into two halves, is usually accompanied by a number of other malformations. One of the cases in this paper appeared to have an epidermoid tumor which is rarely associated with this seldom seen congenital anomaly. Diastematomyelia, which becomes symptomatic in childhood, requires early surgical intervention to relieve the tethering effect on the cord by removal of the splitting pathology which is thought to be the main cause of neurological deterioration in the ensuing years.