BACKGROUND: Primary cardiac tumors in infants and children are extremely rare; hence, there is very little literature available, and most knowledge is based on collections of case reports. This report is a comprehensive review of our 26-year experience with primary cardiac tumors in children with emphasis on surgical indications, strategies, and long-term outcome. METHODS: Between 1986 and 2012, 47 children (mean age 5.9 +/- 2.4 months; range, 1 day to 17 years) underwent either subtotal or total resection of cardiac tumors (rhabdomyoma, 13; fibroma, 12; teratoma, 9; myxoma, 8; hemangioma, 2; rhabdomyosarcoma, 1; non-Hodgkin’s lymphoma, 1; lymphangioma, 1). The majority were diagnosed by echocardiography (n = 33). Clinical patterns were varied: 40 had an atypical heart murmur and 6 were asymptomatic. Outflow tract obstruction of more than 30 mm Hg was present in 11 children. Three patients had abnormal coronary arteries secondary to pressure from tumor bulk. Indications of resection were hemodynamic/respiratory compromise, severe arrhythmia, and a significant embolization risk. Strategy of resection varied according to location and hemodynamic status without damage to adjacent structures. RESULTS: Morbidity included bleeding in a patient and a transient low output state in another. A 5-month-old infant with left ventricular fibroma underwent left ventricular assist device implantation secondary to failure from weaning off cardiopulmonary bypass, and she eventually underwent heart transplantation 17 days later. Early mortality (n = 2, 4.2%) included a 5-month-old infant who underwent complete resection of rhabdomyoma located in the left ventricle, with concomitant pulmonary valve replacement; unfortunately, he underwent left ventricular assist device implantation for postoperative heart failure and died on the 13th postoperative day. An 8-month-old child with 3 cm x 4 cm fibroma obstructing the right ventricular outflow tract compressing the right coronary artery died of severe right-side heart failure on the 13th postoperative day. One late death (2.1%) occurred; a 16-year-old with non-Hodgkin’s lymphoma died 7 months after the surgery. Mean duration of follow-up is 11.6 +/- 3.5 years. All survivors (93.4%) are well, free of tumor-related symptoms and tumor recurrence/progression, even when resection was incomplete. CONCLUSIONS: This study illustrates that although primary cardiac tumors in infants and children have a wide and unusual spectrum of clinical presentation, an individualized approach to tumor resection allows restoration of an adequate hemodynamic function and satisfactory long-term, tumor-free outcome.