We report on an infant with rhabdomyoma diagnosed prenatally by echocardiography which revealed specific patterns of intracardiac tumors. The mother had been previously diagnosed with tuberous sclerosis. Since birth, the infant's intracardiac tumor has regressed and he has shown good growth and development without surgery in more than one year of follow-up. In tuberous sclerosis, a cardiac rhabdomyoma is the only sign that can be detected prenatally by ultrasound. Even though a cardiac rhabdomyoma occasionally induces poor outcome, the need for surgery depends on the patient's clinical presentation.