Von Hippel-Lindau (VHL) syndrome is an autosomal dominant disorder caused by mutations in the VHL tumor suppressor gene, characterized by visceral cysts and neoplasms including pheochromocytomas, renal cell carcinoma (RCC), pancreatic tumors, and central nervous system (CNS) hemangioblastomas, etc (1–3). The gene discussed is VHL; the disease is von Hippel-Lindau disease.