Recent studies have indicated that up to 40% of pheochromocytomas could be attributable to an inherited germline variant in an increasing list of susceptibility genes (6), which can be grouped into three clusters: pseudohypoxia group (VHL, SDHA, SDHB, SDHC, SDHD, SDHAF2, FH and EPAS1), kinase signalling group (RET, NF1, TMEM127, MAX and HRAS) and Wnt signalling group (CSDE1 and MAML3) (7). This evidence concerns the gene SDHB and hereditary pheochromocytoma-paraganglioma.