ABSTRACT
Although cases of von Hippel-Lindau syndrome are not rare, the fact that hemangioblastomans can occur at sites in the nervous system other than the cerebellum is not appreciated. We are reporting a case of VHL complex in 23-year-old male. In this case, there were multiple cerebellar hemangioblastomas, retinal angiomas, multiple spinal hemangioblastomas, syringomyelia, and papillary cystadenoma of epididymis. There was no evidence of polycythe mia or abnormality of other organs. Large cystic cerebellar hemangioblastomas were removed surgically and papillary cystadenoma of epididymis were excised, too. Total laminectomy of T2, T3 and T4 revealed abnormal meningeal varicosities, enlarged spinal cord, inra-axial hemangioblastomas, and syringomyelia.