This study reviewed different types of primary cardiac and mediastinal tumors in infants and children as well as their clinical presentation and management. Altogether, 34 consecutive patients followed from 1976 through December 2005 were analyzed. Of these 34 patients, 14 (41%) underwent surgery and 20 (59%) with rhabdomyomas were managed conservatively. Histologic examination of the surgically resected tumors showed teratoma in four patients, rhabdomyoma in four patients, and hemangioma in two patients. Myxoma, myofibroma, neuroblastoma, and malignant fibrous histiocytoma were encountered in one patient each. Follow-up evaluation was completed for 97% of the patients and extended up to 30 years. Half of the nonsurgical patients with rhabdomyoma showed partial or complete spontaneous regression. One patient died after resection of a malignant histiocytoma, and one patient required a tumor-related reoperation. Freedom from tumor-related reoperation after 10 years was 91% +/- 8.7%. Of the survivors, 85% were New York Heart Association (NYHA) class 1, and 100% had sinus rhythm. Spontaneous tumor regression is common in rhabdomyoma and surgery, and is indicated only for symptomatic patients with hemodynamically significant intracardiac obstruction. For all other benign primary cardiac tumors, complete resection usually can be accomplished with good results. Patients with giant tumor masses compressing or infiltrating the heart frequently cannot undergo complete resection. For these patients, restoration/preservation of sufficient heart function is the primary goal. Malignant tumors are extremely rare in pediatric patients and have a very poor prognosis.