The medical management of women at an increased risk of breast cancer has substantially changed over the past few years, with improvements in screening, cancer prevention, and treatment, and is best defined for women with mutations in BRCA1 and BRCA2. Screening techniques, such as breast magnetic resonance imaging, enable early detection of cancers in high-risk women. Prophylactic salpingo-oophorectomy has been demonstrated to decrease the risk of breast and ovarian cancers, as well as mortality in BRCA1/2 mutation carriers. Most recently, there have been considerable strides in the treatment of cancers in BRCA1/2 mutation carriers. Breast cancer patients with BRCA1/2 mutations treated with systemic therapy do not appear to have excess toxicity, and have similar relapse-free and overall survival compared to noncarriers. Preclinical and early clinical research suggests that specific classes of chemotherapy may be more effective in mutation carriers. PARP inhibitors represent a novel therapeutic strategy that exploits the weaknesses of BRCA1/2-associated malignancies.