&NA;A case of intramedullary cavernous angioma of the upper cervical spinal cord, initially associated with trigeminal neuralgia, is reported. Magnetic resonance imaging precisely depicted the entire extent of the lesion. The angioma was totally removed and the operation was successful in relieving the patient of neuralgia. The previously reported 23 cases of intramedullary cavernous angiomas are reviewed, and the clinical symptoms, diagnosis, and treatment of this rare condition are discussed. (Neurosurgery25:97‐101, 1989)