Giant cell ependymoma (GCE), is a extremely rare subtype of ependymoma. To date 14 cases have been documented in the literature. Here we present a case of GCE occuring in a 32-year-old patient with a 3-month history of progressively increasing pain in low back and right leg. Magnetic Resonance Imaging demonstrated a well circumscribed and enhancing intramedullar mass extending from L1 to L3. Tumor was totally resected. Histological and immunohistochemical findings were consistent with low grade giant cell ependymoma. This case is the first GCE of a lumbar spinal cord.