The spinal intradural extramedullary cavernous angioma is a rare clinical entity. Only 20 surgically treated cases have been reported. The authors report on an additional case in which the lesion was located in the cervical region, and they summarize its unique clinical features. Intradural extramedullary cavernous angiomas occur predominantly in males, in the lower thoracolumbar region, exhibit a relatively high association with subarachnoid hemorrhage, and mostly adhere to the nerve root or spinal cord. Because resection is possible without causing morbidity and because outcome depends on the severity of preoperative neurological dysfunction, precise diagnosis and timely treatment are mandatory.