Hemangioblastomas are rare benign tumor of the central nervous system that commonly occur in the posterior fossa around the 4th ventricle. In case of von Hippel-Lindau disease, hemangioblastomas involve multiple regions such as cerebellum, spinal cord and brainstem but, rarely show simultaneous involvement of cerebellum and spinal cord. We have experienced a case of multiple hemangioblastomas that were located at the cerebellum, cervical cord and conus medullaris and also had multiple lesions that a part of von Hippel-Lindau disease;retinal angioma, syringomyelia, multiple cyst on kidney and pancreas, renal cell carcinoma on left kidney. Hemangioblastomas on cerebellum and spinal cord were removed totally, retinal angioma was treated with laser photocoagulation and renal cell carcinoma was also totally excised. The authors report a case of von Hippel-Lindau disease had multiple located hemangioblastomas on cerebellum, cervical cord and conus medullaris with review of literature.