Abstract Objective To retrospectively summarize the one-stage correction of congenital heart disease with tracheal stenosis Methods From August 2001 to November 2005, six cases of congenital heart disease with tracheal stenosis were corrected by one-stage surgery; the age at surgery ranged from 24 d to 3 years, with a mean of 13.16±12.03 months, and the weight ranged from 4.2 to 10 kg, with a mean of 7.98±2.03 kg, including three cases of tetralogy of Fallot, one of which was associated with pulmonary atresia; 2 cases of ventricular septal defect, 1 of which was also associated with aortic constriction; and 1 case of pulmonary artery sling. All cases underwent correction of precordial disease under hypothermic extracorporeal circulation and simultaneous management of tracheal stenosis. The result was one case of early postoperative death, which could not be disconnected from the ventilator due to homogeneous stenosis of the right and left bronchi. one case of pulmonary artery sling, which was discharged successfully after surgery, developed endotracheal granulomatosis after 3 months, and the family abandoned the treatment. The remaining 4 cases recovered well after surgery and were followed up for 6 months to 4 years with no clinical manifestations of shortness of breath. Ultrasound review of cardiac function was good, and three of them were reviewed by CT, showing a patent tracheal anastomosis with no obvious stenosis. It is not only safer to correct the tracheal stenosis under extracorporeal circulation during surgery, but also beneficial to the rehabilitation of children after surgery by correcting the preconditioning at the same time. The management of pediatric congenital tracheal stenosis is difficult, and different methods can be used depending on the degree of stenosis, but the principles of management include the use of autologous tissue, maintaining the morphology of the trachea and the cellular function of the tracheal lining, and maintaining tracheal growth and good long-term results.