Germline loss-of-function SDH mutations are associated with several tumors such as pheochromocytoma and paraganglioma, gastrointestinal stromal tumor (GIST), and renal cell carcinoma (Hensen & Bayley 2011; Kantorovich et al. 2010; Anderson et al. 2004; Astuti et al. 2001; Miettinen et al. 2011; Badve et al. 2011; Ricketts et al. 2008). The gene discussed is SDHB; the disease is hereditary clear cell renal cell carcinoma.