Variants in the genes BRCA1 or BRCA2 are known to confer a high lifetime risk of developing breast cancer, because the presence of certain heterozygous BRCA variants increases the risk of breast (~80% for BRCA1) and ovarian cancer (~40% for BRCA1), identifying BRCA1 and BRCA2 as high-penetrance breast cancer predisposition genes [3]. The gene discussed is BRCA1; the disease is ovarian carcinoma.