AbstractPulmonary artery sling is an unusual anomaly. The results of surgical therapy have been unsatisfactory in the past because of poor long‐term left pulmonary artery patency and failure to address concomitant primary tracheobronchial stenoses. Refinement of suture materials and microsurgical techniques have allowed earlier surgical treatment of tracheal stenosis and have led to improved pulmonary artery patency rates. Intermediate results of primary one‐stage repair with tracheal resection and left pulmonary artery reimplantation or translocation in early childhood have been promising. It remains to be seen whether late anastomotic tracheal stenosis will be a problem. This constitutes the first report of a case of one‐stage repair with tracheal resection and left pulmonary artery reimplantation in an i