Inherited mutations in BRCA1 and BRCA2 and Lynch syndrome (LS) account for a significant minority (15% to 25%) of ovarian cancers (OCs)1,2 and confer a high risk for OC: 11% to 37% by age 70 years in BRCA2 carriers and 39% to 65% in BRCA1 carriers.3,4 Other lower-penetrance homologous repair pathway genes have been implicated in familial OC.5,6. The gene discussed is BRCA2; the disease is Leigh syndrome.