Germline mutations in the RET, VHL and NF1 genes predispose to phaeochromocytoma in the context of inherited tumour syndromes, including neurofibromatosis, multiple endocrine neoplasia type 2 (MEN 2) and Von Hippel Lindau syndrome (VHL) [18,19]. Here, VHL is linked to von Hippel-Lindau disease.