We reported the case of a 40-year-old man who was hospitalized to our department on November 16, 1975 with a year history of neuralgia in the saddle region and vesicorectal dysfunction. Examination of CSF on lumbar puncture at L4-5 revealed xanthochromia with a protein content of 2,560 mg/dl. Myelography revealed a tortuous filling defect at the level of L2 coupled with complete blockade at L3 level. Selective spinal angiography of the left 12th intercostal artery demonstrated the enlarged Adamkiewicz's artery. This blood vessel was found to enter a diffuse uniform density, where no distinct blood vessels were visible. Within one second after the injection of contrast agent an efferent vein appeared from the right side of the density, ascended to the level of Th12 and then, upon turning, descended to the pelvic canal. These findings suggested a hemangioblastoma of the spinal cord. Laminectomy was performed at levels of L2 through L4 and a hemangioblastoma of the cauda equina was totally excised at L3 level. Although the postoperative course was complicated by a transient dysuria and hypesthesia at S1-S5 levels, complete cure was achieved in a month. In the present paper clinical features and neuroradiological examination of a hemangioblastoma of the spinal cord were described.