Cartilaginous exostosis developed in the atlantoaxial region of a three-and-a-half-year-old Bernese mountain dog. The dog exhibited ataxia in the hindlimbs and flailing movements in the forelimbs. On survey radiographs of the cervical spine there was a focal calcified mass between the dorsal arch of the atlas and the spinous process of the axis. Lumbar myelography revealed severe dorsal spinal cord compression. The mass was removed surgically and the dog made a complete recovery. Histopathology of the excised mass was consistent with a diagnosis of cartilaginous exostosis.