VHL type 1 disease is characterized by development of clear cell renal cell carcinomas and hemangioblastomas, but not PCCs, and is associated with gross deletions in VHL. Type 2 disease is predominantly associated with VHL missense mutations and the development of PCCs (type 2C) or PCCs and hemangioblastomas (type 2A) or PCCs, hemangioblastomas and RCCs (type 2B). Here, VHL is linked to clear cell renal carcinoma.