Mutations in the VHL gene can cause Von Hippel–Lindau syndrome (VHLS), which is a dominantly inherited familial cancer syndrome predisposing to a variety of malignant and benign neoplasms, most frequently retinal, cerebellar, and spinal hemangioblastoma, renal cell carcinoma, pheochromocytoma, and pancreatic tumors. This evidence concerns the gene VHL and hereditary clear cell renal cell carcinoma.