OBJECTIVE: To investigate the surgical management strategy and prognosis of congenital ventricular septal defect (VSD) with mitral regurgitation in infants and children. Methods: From January 2008 to December 2012, a total of 261 children with VSD combined with mitral regurgitation underwent corrective surgery at our center, and were divided into a mitral valve plastic group (n = 139) and an unplanned mitral valve group (n = 122) according to whether mitral valvuloplasty was performed intraoperatively. The postoperative follow-up ranged from 1 to 28 months, with monitoring of ejection fraction and preoperative/postoperative changes in mitral regurgitation. Results: There were no deaths in this group. Mitral regurgitation was reduced in 89 (64.03%), unchanged in 41 (29.50%), and worsened in 9 (6.47%) of the patients in the mitral valve revision group; mitral regurgitation was reduced in 73 (59.84%), unchanged in 37 (30.33%), and worsened in 12 (9.84%) of the patients in the unprocessed group. There was no difference in the change of mitral regurgitation status between the two groups preoperatively and postoperatively. Conclusion: In infants and children with VSD combined with mitral regurgitation, a conservative intraoperative mitral valve management strategy can provide a satisfactory surgical outcome.