Pulmonary artery sling is a rare congenital condition in which the left pulmonary artery arises anomalously fmm the right pulmonary artery extrapericardially and encirdes the right main stem bronchus and passes between the trachea and esophaghus to reach the hilum of the left lung. Respiratory obstruction vesults from compression of the lower area of trachea and right main stem bronchus. It is rare cause of respiratory distress in the new bom and may be associated with significant morbidity and mortality. We experienced the case of pulmonary artery sling with diffuse trachea stenosis and congenital heart disease (pulmonary atresia and PDA) and confirmed the anomaly by bronchogram and MRI. The presumed cause of death was respiratory obsruction due to pulmonary artery sling.