We report a 41-year-old man who presented with low back pain, lower extremity paresthesia, urinary retention and constipation. Magnetic resonance imaging showed a vascular intradural-extramedullary lesion at the second lumbar vertebral level. His medical history revealed that he had undergone surgery for a cerebellar hemangioblastoma 5 years ago. The patient underwent a spinal operation and a vascular tumor was removed from filum terminale. Pathologic examination of the tumor revealed a hemangioblastoma. Hemangioblastomas may occur sporadically or in association with von Hippel-Lindau disease. In the second case, they are often multiple and accompanied by cerebellar and brainstem lesions. The hemangioblastomas reported in the conus medullaris or in the extramedullary compartment adjacent to the conus medullaris are rare, tumors of the cauda equina are uncommon, and lesions of the filum terminale are extremely rare. We report a patient with von Hippel-Lindau disease having filum terminale hemangioblastoma and discuss the diagnosis, pathogenesis and treatment of hemangioblastoma.