In extreme rarity, spinal cord compression may be caused by intradural extramedullary cysts. One of these rare cysts, the ependymal cyst originates, from ectopic ependymal fragments situated parallel to the central canal development and may be seen anywhere in the spinal aids. Considering this rarity and reviewing the literature, we present a spinal cord ependymal cyst case along with a discussion of histopathological and surgical findings.