Cardiac rhabdomyoma is a rare tumor arising from striated muscle. It is a type of hamartoma seen in pediatric population. Solitary rhabdomyomas are unrelated to Tuberous sclerosis. Though most are asymptomatic, few may develop symptoms of congestive cardiac failure. Arrhythmia can occur if the conduction pathway is affected. Hemodynamic instability and progressive disease require surgical intervention. We report a rare case of solitary cardiac rhabdomyoma in a 16 year old female who presented with a large homogenous mass in the right atrium with the involvement of inferior vena cava which was managed surgically with the reconstruction of the inferior vena cava with pericardial patch.