EMSY was first reported to bind BRCA2 and to inactivate the function of BRCA2, leading to the development of sporadic breast and ovarian cancers. The function of EMSY may also be involved in DNA damage repair, genomic instability, and chromatin remolding. Recent studies have shown that amplification of EMSY was also associated with other cancers such as prostate and pancreatic cancers and linked to tumor phenotypes and clinical outcomes. By reviewing literatures published since 2003, here, we have summarized the recent advances of EMSY in cancer development.