While available evidence suggests that nonsense and frameshift alterations in BRCA1 exon 11 confer an increased risk for cancer, the risk profile and clinical management guidelines for heterozygous individuals may be attenuated relative to other complete loss-of-function alterations in BRCA1. Further study of the genotype-phenotype relationship for heterozygous carriers of BRCA1 exon 11 nonsense and frameshifting variants is warranted to help inform clinical care. Here, BRCA1 is linked to cancer.