A dog presented with a 1‐month history of left‐sided hemiparesis. MRI showed a focal, 4‐cm‐long, symmetrical, ovoid, poorly demarcated intramedullary expansion at C6–C7 that was T2‐weighted hyperintense, T1‐weighted isointense, and noncontrast enhancing. After clinical progression and euthanasia, pathology revealed a neoplasm composed of astrocytes and dysmorphic neurons, consistent with a ganglioglioma. The diagnosis was confirmed with immunohistochemistry and transmission electron microscopy, which demonstrated electron‐dense granules in the perikaryon. Gangliogliomas are rare, benign neoplasms that may present as intramedullary spinal cord neoplasia. This is the first report on the clinical presentation, imaging, and pathology of a canine spinal ganglioglioma.