Clear cell variant of ependymoma is almost exclusively located in the supratentorial region. Only few cases of this tumor that located in the spinal cord have been reported. Here we report one case of intramedullary clear cell ependymoma of the lumbar spinal cord. In microscopic examination, the tumor cells were round to oval with moderate amounts of clear cytoplasm and centrally located large nuclei, resembling oligodendroglioma. Typical features of ependymoma, such as ependymal clefts, perivascular pseudorosettes, as well as nuclear pseudoinclusions and grooves were identified. Albeit being rare, clear cell ependymoma could be mentioned in differential diagnosis of clear cell tumors in this area.