We report a rare case of spinal intramedullary ependymal cyst in a 44-year-old female and reviewed 12 cases reported in the literature. The patient presented with slowly progressive lower limb paresis. She underwent biopsy of the cyst wall and placement of a cysto-subarachnoid shunt with complete recovery at the follow-up examination 18 months after surgery. This is a benign lesion and appropriate management should be performed at an early stage of the disease.