Breast magnetic resonance imaging (MRI) has been proposed as an additional screening test for young women at high risk of breast cancer in whom mammography alone has poor sensitivity. We conducted a systematic review to assess the effectiveness of adding MRI to mammography with or without breast ultrasound and clinical breast examination (CBE) in screening this population. We found consistent evidence in 5 studies that adding MRI provides a highly sensitive screening strategy (sensitivity range: 93-100%) compared to mammography alone (25-59%) or mammography plus ultrasound+/-CBE (49-67%). Meta-analysis of the three studies that compared MRI plus mammography versus mammography alone showed the sensitivity of MRI plus mammography as 94% (95%CI 86-98%) and the incremental sensitivity of MRI as 58% (95%CI 47-70%). Incremental sensitivity of MRI was lower when added to mammography plus ultrasound (44%, 95%CI 27-61%) or to the combination of mammography, ultrasound plus CBE (31-33%). Estimates of screening specificity with MRI were less consistent but suggested a 3-5-fold higher risk of patient recall for investigation of false positive results. No studies assessed as to whether adding MRI reduces patient mortality, interval or advanced breast cancer rates, and we did not find strong evidence that MRI leads to the detection of earlier stage disease. Conclusions about the effectiveness of MRI therefore depend on assumptions about the benefits of early detection from trials of mammographic screening in older average risk populations. The extent to which high risk younger women receive the same benefits from early detection and treatment of MRI-detected cancers has not yet been established.