The lifetime risk for breast cancer in patients with BRCA1 or BRCA2 mutation is 60–85% but for ovarian cancer is 40–60% in BRCA1 patients versus 10–30% in BRCA2 patients. The risk of contralateral breast cancer is 30% within 5 years of the first breast cancer. Risk-reduction options include either bilateral mastectomy with (95% reduction) or without (90% reduction) oophorectomy, oophorectomy alone before the age of 50 years, or tamoxifen chemo-prevention (38% risk reduction). Other options include more intense and earlier screening starting at the age of 30 years with annual MRI of the breast. The role of mammography in BRCA1 mutation carriers is controversial due to an increased risk of radiation-induced breast cancer in vitro. Compared with MRI as a screening method alone, the majority of comparative imaging screening trials shows a small number of additional cancers with mammography but no or very little value of US after annual MRI and mammography [4, 5]. Imaging features of breast cancer in high-risk women are often more benign appearing, particularly in BRCA1 mutation carriers, in whom 23% of invasive ductal cancer demonstrates a fibroadenoma-like appearance with an oval or round shape and smooth margins but no dark septations [6]. Moreover, no mammographic calcifications are seen in invasive cancer. A posterior prepectoral location of breast cancer is seen in 67% of BRCA1 mutation carriers [6]. Second-look US is crucial to identify suspicious lesions on MRI to allow subsequent USguided biopsy. If US remains negative, spot-compression mammography or tomosynthesis may help identify MRI lesions. If no traditional imaging method identifies the suspicious MRI-only lesion, then MRI-guided biopsy with clip positioning and postbiopsy mammography allows adequate patient management. A 6-months’ follow-up, MRI remains important to demonstrate no increase in size after a negative MRI-guided biopsy. The benefit of high-risk screening was shown in a study comparing breast cancer in BRCA1 and BRCA2 patients diagnosed with and without MRI. The group with MRI had significantly smaller tumors and less chemotherapy; however, the slightly higher 3-year and disease-free and overall survival was not significant [7].