We report on a case of a newborn with echocardiographic diagnosis of anomalous origin of the right coronary artery from the main pulmonary artery. Intraoperative inspection demonstrated a proximal anomalous course of the right coronary artery but a normal origin from the right sinus of Vansalva of the Aorta. We suggest a complete investigation, including coronary angiography, when isolated and rare congenitaj malformations of the coronary arteries are suspected.