CRISPR/Cas genome editing technology is a newly developed powerful tool for genetic manipulation, which can be used to manipulate the genome at specific locations precisely, to restore the function of genetic defect cells, and to develop various disease models. In recentl years, with the advances of precise genome manipulation, CRISPR/Cas technology has been applied to many aspects of diseases research and becomes an unique tool to investigate gene function and discover new therapeutic targets for genetic diseases. Nowadays, CRISPR/Cas technology has been a hot research point in agriculture, graziery, biotechnology and medicine. This review focuses on the recent advances in CRISPR/Cas technology and its application in hematological diseases.