An 8-year and 6-month-old female Maltese dog showed a stoop with rigidity of her cervix and back. Neurologic examination showed loss of proprioception, and deficiency of pain response. Postmortem examination revealed the neoplastic mass replacing the central area in the cervical spinal cord at the level from 4th to 5th segments. Histologically, the mass was composed of neoplastic ependymal cells. The neoplastic cells showed marked atypism, and occasionally formed ependymal rosettes. Based on the morphologic features, the tumor was diagnosed as anaplastic ependymoma. Immunohistochemistry showed that the neoplastic cells were negative for glial fibrillary acid protein, and slightly positive for vimentin and cytokeratin.