Up to 40% of pheochromocytomas are hereditary and caused by germline mutations in well-known cancer susceptibility genes (SDHx, VHL, EGLN1/PHD2, EPAS1/HIF2A, KIF1Bβ, MAX, MEN1, NF1, RET, TMEM127, RAS, NF1, FGFR1, ATRX, etc.)which are involved in interconnecting pathways [1,2]. The gene discussed is EPAS1; the disease is pheochromocytoma.