Tumors are rare in neonates. The most common type is a benign rhabdomyoma. Rhabdomyoma is self-regressing with time. However, if a tumor is huge and causing hemodynamic instability, surgery is indicated. We present successful cardiac surgery for partial removal of a giant rhabdomyoma tumor in the right ventricle of a 7-day-old neonate and spontaneous regression of the remnant tumor mass at follow-up.