The presence of familial or clinical criteria for a phaeochromocytoma-associated or paraganglioma-associated inherited disease should lead to targeted genetic testing. In the absence of criteria, SDHB immunohistochemistry is indicated. A positive SDHB immunohistochemistry result should lead to VHL and RET genetic testing, a negative SDHB immunohistochemistry should lead to SDH (SDHD, SDHB, and SDHC) genetic testing, starting with SDHD in cases of head and neck paraganglioma or starting with SDHB in cases of thoracic–abdominal or pelvic paraganglioma.