Introduction:
Cardiac tumors in children are rare, with the majority being benign primary tumors like rhabdomyoma and fibroma.Malignant tumors of the heart are extremely rare in children, with the majority being sarcomas followed by lymphomas.However, unlike adults, metastatic cardiac tumors are less common than primary malignant tumors of the heart in children. Children with cardiac tumors can be asymptomatic or may present with chest pain, murmur, arrhythmia, heart failure, or sudden death, depending on the location and size of the tumor [1]. We report a very rare case of primary rhabdomyosarcoma of the heart presenting with a life threatening pericardial effusion, and massive local tissue infiltration.