Abstract： Ebstein anomaly is a relatively rare congenital heart malformation which can affect heart function significantly. It may cause right heart failure, even whole heart failure and eventually death. In recent years, the surgery has made much progress in dealing with the abnormal valve, improving the right ventricle function and pretreatment of its related complications. However, because of its complexity and diversity in pathological anatomy and clinical manifestations, the disease has not got an “almighty standard” to treat all pathological anatomy types of the deformity, or asurgery strategy to solve the practical problems encountered in all clinic situations. Furthermore, the therapeutic effect is also unsatisfactory. This article will review the advance of treatment of Ebstein anomaly and prevention of its related complications.