Abstract: @@ Von Hippel-Lindau (VHL) disease is a rare autosomal dominant disorder caused by germ line mutations of the VHL tumour suppressor gene. It predisposes affected individuals to develop a variety of neoplasms, including haemangioblastomas of the central nervous system,retinal angiomas, renal cell carcinomas (RCCs),pheochromocytomas and cysts of the kidneys and epididymis.