A two-month-old female infant was diagnosed with pulmonary artery sling and tracheal stenosis due to postnatal wheezing. She was admitted to our hospital and scheduled to undergo left pulmonary artery reconstruction, tracheoplasty, and ventricular septal defect (VSD) repair. Pulmonary artery sling refers to the abnormal route of the left pulmonary artery, which comes from the back of the right pulmonary artery and forms a vascular ring as it crosses the right main bronchus in a semi-circular manner and passes in front of the esophagus and behind the trachea to reach the left lung hilum. Approximately 50% of children with pulmonary artery sling have other cardiovascular and tracheal malformations, such as congenital tracheal stenosis. The abnormal routed left pulmonary usually can cause tracheal compression and result in airway obstruction, respiratory distress, and even life-threatening situations in severe cases. Preoperative selection of appropriate endotracheal tubes based on whether the child has tracheal stenosis and the degree of compression, rational intraoperative anesthetic management, and correct postoperative airway management are the key to successful anesthetic management and surgery.