The germline or somatic alteration in nuclear mitochondrial complex II genes encoding SDH subunits (SDHB, C, and D) were found associated with pheochromocytomas and paragangliomas (Gimm et al., 2000; van Nederveen et al., 2007; Bardella et al., 2011). The gene discussed is SDHB; the disease is pheochromocytoma.