Additionally to the genes involved in the classical syndromic forms: VHL gene in von Hippel-Lindau disease, RET gene in Multiple Endocrine Neoplasia type 2 (MEN 2), and NF1 gene Neurofibromatosis type 1, 10 novel genes have so far shown to be implicated in the occurrence of paragangliomas/pheochromocytomas [27–29]. Here, VHL is linked to pheochromocytoma.