Although approximately 14 confirmed genes are associated with pheochromocytoma and paraganglioma susceptibility, the genes investigated in the present case – SDHB, SDHC, SDHD, VHL and MAX – have been the main reported genes associated with malignant PCC reported so far, according to the Endocrine Society Clinical Practice Guideline (1). Here, VHL is linked to hereditary pheochromocytoma-paraganglioma.