Intradural extramedullary cavernomas are rare vascular malformations of the spine. Of the 40 previously described cases, four involved the upper thoracic spine and one was associated with familial multiple cavernoma syndrome. We report the case of a 55-year-old man with familial multiple cavernomas presenting with thoracic radiculopathy and back pain due to a T3-T4 intradural extramedullary cavernoma compressing the spinal cord and dorsal nerve rootlets. Vascular malformations of the thoracic spine are a rare cause of atypical chest pain that should be considered in an individual with familial multiple cavernoma syndrome.