Young women who carry a BRCA mutation face difficult decisions regarding radiologic screening modalities and prophylactic surgery. Decisions regarding these choices may have short and/or long-term consequences and significant impacts on breast cancer risk. A comprehensive review of currently available data supports the following recommendations for young women with BRCA mutations: perform monthly self breast exam, obtain clinical breast exam 2-4 times per year, discuss annual imaging options with a breast specialist, complete child-bearing by 35 with subsequent prophylactic oopherectomy, avoid hormone replacement therapy, and consider prophylactic mastectomy.