OBJECTIVE Infective endocarditis is rare in children but potentially carries high mortality and morbidity. Few data exist regarding surgical therapy and the associated outcomes in children with infective endocarditis. The aim of the present study was to describe the characteristics and outcomes of children undergoing surgery for infective endocarditis. METHODS A retrospective review of all patients aged 21 years or younger diagnosed with definitive infective endocarditis at a single center from 1996 to 2010 was performed. RESULTS Of 76 identified patients with infective endocarditis (median age, 8.3 years; 73.9% boys), 46 patients (61%) required surgical intervention. Staphylococcus aureus was most commonly isolated (18 patients, 24%) followed by Streptococcus (17 patients, 22%). Common surgical indications included severe valvular insufficiency in 13 patients, septic embolization in 12, concomitant severe valvular insufficiency and ventricular dysfunction in 9, persistent vegetations in 9, and persistent bacteremia in 3. Although early surgery was performed within 7 days of diagnosis in 35 patients (76%), 25 (54%) underwent surgery within 3 days or less. The factors associated with surgery included the presence of ventricular dysfunction, left-sided vegetation, severe valvular insufficiency, septic embolization, and S aureus. Surgery within 3 days or less was associated with the presence of ventricular dysfunction and S aureus. Native valve repair was performed in 50% of patients with native-valve disease. Postoperatively, no septic embolization events occurred and recurrence was low (2%). The 1-, 5-, and 10-year survival was 98% ± 2%, 90% ± 8%, and 81% ± 11%, respectively. CONCLUSIONS Children with infective endocarditis can undergo successful early surgical therapy with a low risk of septic embolization, recurrence, and operative mortality.