The familial PCC/PGL are associated with germline mutations in at least 11 susceptibility genes, including multiple endocrine neoplasia 2A and 2B (RET), von Hippel-Lindau (VHL), neurofibromatosis type 1 (NF1), transmembrane protein 127 (TMEM127), MYC-associated factor X (MAX), fumarate hydratase (FH), and succinate dehydrogenase complex subunit A, B, C, D, and AF2 (SDHA, SDHB, SDHC, SDHD, SDHAF2) [3–13]. Here, FH is linked to adrenal gland pheochromocytoma.