Von Hippel-Lindau (VHL) disease is a hereditary condition caused by mutations in the VHL gene that lead to the development of vascular tumors such as clear cell renal cell carcinoma (ccRCC), central nervous system (CNS)-hemangioblastoma, and pancreatic neuroendocrine tumors (panNET). The gene discussed is VHL; the disease is nonpapillary renal cell carcinoma.