Germline inactivating SDHB pathogenic variants were first identified in kindreds with familial pheochromocytoma and paraganglioma (PPGL).1 Paragangliomas (PGL) and pheochromocytomas (PCC) are rare neuroendocrine tumours arising from the sympathetic/parasympathetic ganglia and the adrenal medulla, respectively. The gene discussed is SDHB; the disease is paraganglioma.