Pulmonary artery sling is a rare and potentially lethal vascular anomaly wherein the anomalous left pulmonary artery arises from the posterior aspect of the right pulmonary artery and passing to the left lung between the trachea and the esophagus which causes respiratory distress in infants and children due to the extrinsic compression of the airway by the anomalous vessel. Associated anomalies are common, particularly those of the tracheobronchial tree. The most common lesions are hypoplasia of the distal trachea and right main stem bronchus. About 50 percent of patients have cardiovascular anomalies, such as in the left superior vena cava, atrial septal defect, ventricular septal defect, patent ductus arteriosus, aortic arch anomalies, tetralogy of Fallot 1 etc. We experienced a case of a 6-year-old boy who showed dyspnea and frequent pneumonia. He was diagnosed with pulmonary artery sling associated with left bronchial stenosis, patent ductus arteriosus, and left superior vena cava which was parven by echocardiographic examinations, spiral computerized tomography of the chest and cineangiography. We report this case with related literature.