BACKGROUND: A 61-year-old man was admitted complaining of myelopathy and back for 3 months. CASE DESCRIPTION: A 15-mm hemangioma with calcification was noted on magnetic resonance imaging and computed tomography. Intraoperatively, the mass was hard with ill-defined demarcation. The cranial portion of the mass was brown and dark gray on the dorsal cord. During surgery, motor evoked potentials transiently decreased, but they fully recovered at the end of surgery. After surgery, the patient's symptoms recovered without severe neurologic deficit. CONCLUSIONS: Hemangioma calcificans, a variant of cavernous hemangioma with full calcification and ossification, is an extremely rare disease in the spine and brain. Here we report a rare case of intramedullary cavernous hemangioma with calcification of the spinal cord.