Cardiac rhabdomyoma is the most common primary heart tumor in childhood.
This tumor, which is frequently associated with tuberous sclerosis
complex, mostly disappears in childhood with spontaneous regression.
Surgical resection is required in case of outflow obstruction,
arrhythmia and protruding enough to disrupt the filling of the heart
cavities. There are very few case series in the literature about
rhabdomyom, whose relationship with other congenital heart defects has
not been clearly demonstrated. In this case of tuberous sclerosis we
presented to you, we reported our approach to the tumor during the
corrective surgery of the infant, who was diagnosed with
atrioventricular septal defect and patent ductus arteriosus, and
rhabdomyoma accompanying these malformations.