The purpose of this article is to overview the existing breast cancer screening guidelines for women at high risk from world-leading specialty societies. Accumulation of evidence and development of accessible genetic testing strategies have changed the idea of breast cancer screening for high-risk women. Personalized tailor-made screening adjusted for risk factors has been conducted in accordance with guidelines. The use of imaging modalities other than mammography including contrast-enhanced MRI and other various strategies for improving screening are discussed. The present review also mentions the existing challenges in high-risk screening and the latest information based on two large-scale studies.