Germline mutation of the succinate dehydrogenase subunit genes, SDHA, SDHB, SDHC or SDHD, is associated with increased risk for paraganglioma (PGL), pheochromocytoma (Pheo), gastrointestinal stromal tumor (GIST) and renal cell carcinoma [15–18]. The gene discussed is SDHB; the disease is hereditary clear cell renal cell carcinoma.