Rhabdomyoma is the most common intracardiac tumor in the newborn. The diagnosis is made by the clinical history and the physical examination, the electrocardiogram, chest X-ray, and the echocardiographic data. It has a poor prognosis, 80% dying in the first year of life because congestive heart failure or sudden death. Rarely, spontaneous regression has been described. We present the case of a newborn diagnosed of tuberous sclerosis, with an echocardiographic image of a rhabdomyoma in the right ventricle with a spontaneous total regression of the tumor 2 years later.