Spinal intradural extramedullary ependymal cyst is a very rare entity with only few cases reported in the literature. Its association with congenital dermal sinus has not been described so far. We present a unique report of a 3-year-old male child who presented with spastic quadriparesis with a trophic ulcer in the right great toe of 1-year duration. He harbored a congenital dermal sinus in the cervical spine since birth. Intraoperatively, the sinus was associated with an intradural cyst which proved to be an ependymal cyst on histopathological examination. The clinical profile along with review of literature of this rare entity is presented.