Women with BRCA1 or BRCA2 mutations are at substantial risk for breast and ovarian cancer. This review describes recent developments in the approach to hereditary breast cancer. Risk-reducing surgeries remain the most effective means of preventing breast cancer in mutation carriers. For women with breast tissue at risk, magnetic resonance imaging is rapidly becoming incorporated into screening programs. For affected women, management does not currently differ from that of women with sporadic breast cancer, although women may choose to undergo bilateral mastectomy. Preclinical data suggest that BRCA mutation-associated breast cancers may benefit from specific targeted therapeutic approaches.