Primary cardiac tumors are rare in children, usually consist of benign tumors like rhabdomyomas and fibromas that may spontaneously regress. Primary malignant tumors are extremely rare even in adults and very few paediatric cases were reported in literature. Rhabdomyosarcoma is a rare primary malignant tumor in children and most of the reported cases occur in right ventricle, left atrium and right atrium. We report a 15 month old child with primary rhabdomyosarcoma of left ventricle presenting in cardiac tamponade and circulatory failure.