We report the case of a young patient with a thoracolumbar (T11-L3) intramedullary angiolipoma. Total removal of the tumor was possible and after operation the patient's neurological condition greatly improved. Only two previous cases of intramedullary angiolipomas have been reported, and in neither could the lesion be wholly removed. The histopathological and radiological features of these tumors are discussed and the extreme rareness of this case is emphasized.