Intramedullary ependymomas are rare tumors but comprise the majority of intramedullary glial neoplasms in the adult. These tumors are benign, slow-growing lesions which are optimally treated with gross-total surgical resection without adjuvant therapy. This objective can be attained safely in a majority, of patients. Post-operative functional outcome is related to pre-operative functional status. Hence, early diagnosis, prior to symptomatic progression, is critical to the successful treatment of these tumors. Adjuvant therapy is indicated for the rare malignant or disseminated tumor or following sub-total resection.