Although more than 15 years have elapsed since the discovery of the BRCA1 and BRCA2 genes and the associated increased risk of breast and ovarian cancers in mutation carriers, our understanding of the syndrome is still evolving. With the accumulation of knowledge, more questions arise regarding the proper approach to mutation carriers diagnosed as having cancer. Moreover, the number of questions regarding the recommended management methods for healthy carriers and the potential risk-reducing measures is increasing constantly.In this review, we discuss these issues and summarize contemporary recommendations.