This paper reported surgical treatment on 154 patients with CMVI. Of them, pure CMVI only existed in 23 cases, the associated anomalies in 131 cases. Surgical repair of mitral valve were performed in 144 cases, replacement of valve were done in 10 cases. Surgical repair included plication of the commissure in 125 cases, closure of the fenestration of the large leaflet in 1 case, partial resection of the small leaflet with approximation and valve ring annuloplasty in 3 cases, shortening of elongated chordae taeniae in large leaflet in 4 cases, respectively. The smallest age within all patients undergoing mitral replacement was 3 years. There were 1 early and late death, respectively, in this group. However, reoperation were performed in 2 cases for unsatisfactory repair and 1 case for failure of prosthetic valve. The results showed that echocardiography is a simple and effective tool in preoperative diagnosis and making selection of operative indication, finally, leading to proper repair of associated anomalies. Surgical repair on cases of CMVI should be the first choice, and proper procedure should be selected according to the type of mitral valve deformity. The results were satisfactory even without artificial valve ring. Valve replacement was considered to be essential on severe patients and unsatisfactory repair. On children, slight large valve with double leaflets was preferable.