OBJECTIVE: To study the clinical features of hemangioblastomas of the central nervous system (CNS) when they occucr as a manifestation of von Hippel-Lindau's disease (HLD). METHODS: 174 patients with hemanioblastomas of CNS were reviewed from 1982 to 1995. All patients were verified pathologically. RESULTS: Ten of the patients definitely had HLD. Four patients reported a positive family history. Other 5 patients had retinal angiomas. The following lesions were detected: renal cysts(2 patients), renal tumor (1). Five patients were suspicous. CONCLUSION: Hemangio-blastoma may arise in isolation or as a manifestation of von Hippel-Lindau's disease, and is often multiple in the latter. Enhanced MRI can decrease chances of negative diagnosis for tumor nodules. Microsurgery may be helpful to discover unclear lesions. Patients, especially those with HLD, should be subjected to follow-up and regular MRI examinations for a long time.