The authors report a case of oncocytoma arising from the spinal cord in a 40-year-old woman who presented with the complaints of gradual difficulty in walking. The excised tumor was exclusively composed of polygonal cells with abundant homogeneous eosinophilic cytoplasms. Electron microscopy study showed densely packed swollen mitochondria and frequent desmosomes. The histological and ultrastructural findings were consistent with a diagnosis of oncocytoma. To the authors' knowledge, this represents the first reported case of oncocytoma of the spinal cord.