Several cancers including colorectal, glioma, pheochromocytoma, neuroblastoma, hepatic carcinoma, renal carcinoma and non‐small cell lung carcinomas are associated with the molecular deregulation of EPAS1.4, 5, 6, 7, 8, 9, 10, 11, 12, 13. Here, EPAS1 is linked to hereditary pheochromocytoma-paraganglioma.