Primary Undifferentiated pleomorphic sarcomas (UPS) of the heart extremely rare but aggressive tumors in the pediatric age group with an extremely poor prognosis and no clear guidelines available for best management. Diagnosis is often delayed, as they may be insidious and masquerade as other cardiac benign tumors. We present a rare case of cardiac UPS in a 13 year old with 40 months disease free survival following a combination of early gross total surgical resection followed by adjuvant chemotherapy and radiation therapy. The case highlights the importance of a timely diagnosis and multimodal multidisciplinary aggressive approach for improved survival.