A 4-year-old Labrador Retriever presented for urinary incontinence and constipation of 2 weeks duration. There was a tender abdomen, lumbar pain and conscious proprioceptive deficits in both pelvic limbs. Depressed pelvic limb reflexes were present consistent with a lower motor neuron lesion. In radiographs of the lumbar spine there was narrowing of the intervertebral disc space at L5–L6 with irregular, multifocal areas of mineralized opacities dorsal to the intervertebral disc space, presumably within the vertebral canal. On computed tomography, an intramedullary, partially mineralized mass was identified in the spinal cord at the level of caudal Ls through cranial L6. At necropsy there was a four-centimeter enlarged, irregular segment of spinal cord at the level of L5–L6. When sectioned, the spinal cord had a mineralized texture. Histologically there were variable sized cells that were stellate in appearance with vacuolated cytoplasm (physaliferous cells) and mucinous background consistent with a chordoma. Chordoma is a rare, skeletal neoplasm that originates from mesoderm-derived notochord and has been reported in humans and animals. Extraskeletal development of a chordoma within the spinal cord is a rare manifestation of this neoplasm. However, based on other reports in dogs, solitary extraskeletal locations of chordomas may be the typical expression of this neoplasm in the dog. Differentiation of similar histologically appearing tumors, such as a parachordoma or myxoid chondrosarcoma, will require immunohistochemical characterization of these tumors in veterinary patients.