Subependymoma is a rare benign CNS tumor (WHO grade I). The common sites of origin for this type of tumor are the lateral and the fourth ventricles. A spinal intramedullary location is rare. Radiological features of intramedullary subependymoma can mimic more common and more aggressive tumor types. We report a case of a 61-year-old male who presented with a 3-year history of low back pain, associated with right lower limb progressive weakness and sensory numbness. An MRI of the lumbar spine revealed an intradural intramedullary lesion extending from the level of T10-L1. Clinical presentation, radiological, and pathological studies of this case of subependymoma are presented. The incidence of spinal subependymoma was also discussed in light of a literature review.