VHL and hemangioblastoma: Clinically, two presentations are observed: (i) an individual with a family history of VHL and the presence of a CNS or retinal hemangioblastoma, a pheochromocytoma or a clear cell renal cell carcinoma; or (ii) an index case (an individual with no family history) with two or more hemangioblastomas or two or more visceral tumors or one hemangioblastoma and one visceral tumor [4].