Mutation of the von Hippel-Lindau (VHL) tumor suppressor gene is associated with a hereditary cancer syndrome called von Hippel-Lindau (VHL) disease, which is characterized by an increased risk of clear cell renal carcinoma, hemangioblastoma of the nervous system, and adrenal pheochromocytoma (for reviews see [1]–[4]). The gene discussed is VHL; the disease is clear cell renal carcinoma.