Abstract Background Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital heart disease, characterized by the coronary artery inappropriately originates from the aorta. It is usually classified according to the sinus where the coronary artery arises from, while anomalous origin of the right coronary being the most common type. Case presentation In this case report, we described a rare case of Tetralogy of Fallot (TOF) in a 1-year-old boy, who also had the anomalous right coronary artery that originated from the left coronary sinus without an intramural segment. Besides TOF repair, lateral pulmonary translocation was undertaken in order to avoid risks of myocardial ischemia. Conclusion We successfully completed a one-stage operation consisting of TOF repair and pulmonary artery translocation in a 1-year-old boy. We advocated early operation of pulmonic translocation for AAOCA patients without an intramural segment instead of unroofing procedure.