Several genes are known to play an important role in the pathogenesis of pheochromocytoma, including the RET proto-oncogene, von Hippel–Lindau disease tumor suppressor (VHL), neurofibromatosis type 1 tumor suppressor (NF1), genes encoding the succinate dehydrogenase (SDH) complex subunits, TMEM127, and MYC-associated factor X (MAX) [7]. Here, VHL is linked to hereditary pheochromocytoma-paraganglioma.