The majority of PCC/PGL occur sporadically but although only about 10% of cases have a family history, more than a third of cases harbour a germline mutation in one of the 12 inherited PCC/PGL genes (FH, HIF2A, MAX, NF1, RET, SDHA, SDHB, SDHC, SDHD, SDHAF2, TMEM127, and VHL) that have been shown to be mutated in multiple families and some of these genes are also somatically mutated in sporadic PCC/PGL [1–8]. The gene discussed is VHL; the disease is adrenal gland pheochromocytoma.