We describe the case of a 2-month-old infant with the rare association of aortopulmonary window and anomalous origin of the right coronary artery from the pulmonary artery. The diagnosis was made by angiography and surgical repair was carried on employing the Takeuchi procedure. The angiographic control obtained 5 years after the operation showed an optimal surgical result. The latest echocardiographic follow-up, 24 years after the repair, has been fully satisfactory.