Tetralogy of fallot is rarely associated with anomalous left coronary artery connection to main pulmonary artery. High index of suspicion is needed preoperatively to diagnose this association and treat successfully. We present a case of 9-year-old boy with the rare association of tetralogy of fallot and anomalous left coronary artery connection to pulmonary artery with a giant steal intercoronary collateral crossing right ventricular outflow tract who was treated successfully by single-stage surgical correction.
