Tumorigenic mutations in the von Hippel-Lindau (VHL) gene are associated with the VHL syndrome, an autosomal dominant disorder, which increases susceptibility to various tumors, both benign and malignant, including central nervous system haemangioblastomas, renal cysts and renal cell carcinoma (RCC) and phaeochromocytoma [1], [2], [3]. This evidence concerns the gene VHL and von Hippel-Lindau disease.