Background: Children with infective endocarditis (IE) have to undergo valve replacement instead of valve repair in China due to severe valve damage. The present study is to review our experience on surgical treatment of children with IE in reference to the incidence, pathologic status, diagnosis, surgical strategies and outcomes. Methods: We reviewed 35 patients with a mean age of 13.7±2.2 years who were underwent valve replacement surgery for IE during the period from January 1993 to December 2013. Preoperative transthoracic echocardiographic (TTE) evaluation and transesophageal echocardiography during operation were performed in all patients. All the children underwent chart review and retrospective risk-hazard analysis. Results: Among the patients surveyed congenital cardiac lesions were present in 15 (42.8%), rheumatic heart valve disease in 2 (5.7%) and previous heart surgery in 2 (5.7%). The median stay of intensive care unit was 6 days. Intraoperative findings showed that the endocarditis involved mostly the mitral and aortic valves (88.5%). Triple or quadruple valve involvement was found in one patient each. Ten-year freedom from IE-related death and re-intervention was 94.2% and 91.6%, respectively. Conclusions: Children undergoing surgery for IE frequently have advanced disease with embolic complications. Although valve replacement is not the primary option for pediatric IE, the rate of 5-year survival and freedom from re-operation was optimal prognostically. Pediatric physicians should pay attention to the common clinical features of IE so that the native valve is preserved well.