A 6-year-old male Pointer dog was presented with a 4-week history of progressive hind-limb stiffness. Magnetic resonance imaging demonstrated a focal intramedullary lesion at T1 level with a pattern of ring contrast enhancement. At necropsy, a circumscribed intramedullary reddish-gray tumor was observed. Microscopically, the tumor was composed of thin-walled capillaries lined by endothelial cells and separated by pleomorphic cells (stromal cells) with a moderate degree of anisokaryosis. Immunohistochemically, the endothelial cells were positive for factor VIII-related antigen and the stromal cells were positive for neuron-specific enolase and vimentin. GFAP-positive astrocytes were occasionally observed within the tumor. Both endothelial and stromal cells were negative for synaptophysin, S-100 protein, pankeratin, smooth muscle actin, CD34, CD68, alpha1-antichymotrypsin, and lysozyme. The tumor showed considerable morphologic and immunohistochemical similarities with human hemangioblastoma, and hence the inclusion of this tumor type within the primary neoplasms of the canine central nervous system is suggested.