About one-third of PCC occur in a hereditary context and are characterized by germline mutations in key susceptibility genes including the RET and HIF2A proto-oncogene and the VHL, NF1, SDHx (SDHA, SDHB, SDHC, SDHD, SDHAF2), FH, TMEM127 and MAX tumor suppressor genes (TSGs) [1-11]. The gene discussed is VHL; the disease is adrenal gland pheochromocytoma.