Congenital dermal sinuses result from a failure of separation of the cutaneous epithelial ectoderm from the neuroepithelial ectoderm along the dorsum of the embroy during the first month of intra-uterine life. These sinus tracts can occur at any level of the cerebro-spinal axis, but are located predominantly in the occipital and lumbosacral regions and occasional cases involving the cervical and thoracic regions. The dermal sinus tract is lined by stratified squamous epithelium and extends from the surface through the deeper tissues into the cranial or spinal cavity, usually ending on the dura or within the dura in conjunction with a terminal epidermoid or dermoid cyst. An intra-medullary epidermoid or dermoid with a dermal sinus is an unusual association. We have been able to find a previously reported coincidence of these malformations although there are many definitive papers on these uncommon entities. We are experienced in one case who are 14 months old boy with a intra-medullary dermoid cyst associated with a congenital dermal sinus at the T4 level, and reviewed related reports.