The normal VHL gene is a tumor suppressor gene and it seems likely that the diminution of tumor suppression activity which results from the mutation of that gene leads to the various manifestations of the disease: renal cysts and renal carcinoma, pheochromocytoma, pancreatic cysts, neuroendocrine tumors, cystadenomas of the reproductive adnexal organs, and hemangioblastomas of the cerebellum, spinal cord, brain stem, and retina. The gene discussed is VHL; the disease is hemangioblastoma.