VHL is mainly known for its role in the VHL syndrome, a disease caused by a mutation in the VHL gene on the short arm of chromosome 3, and this syndrome is associated with the development of various malignant diseases, including hemangioblastoma of the retina and of the central nervous system, and with tumours as renal cell carcinoma (RCC) and pheochromocytoma [12]. The gene discussed is VHL; the disease is hereditary pheochromocytoma-paraganglioma.