SDHB is the most commonly mutated of all the SDH-related genes, with >180 mutations that have been associated with extra adrenal, head or neck paraganglioma, adrenal pheochromocytoma, Carney–Stratakis syndrome, renal cell carcinoma, GIST, and infantile leukodystrophy. Here, SDHB is linked to hereditary clear cell renal cell carcinoma.