Severe tracheal stenosis is a rare life-threatening condition that often requires early surgical intervention. The management of this anomaly has been associated with significant mortality and morbidity. We describe our experience with repair of this condition. From 1986 to 2011, 20 patients underwent repair of tracheal stenosis at the Royal Children's Hospital (median age 4.9 months) and were retrospectively reviewed. Tracheal repair techniques used were as follows: patch tracheoplasty (n=8; 40%, 8 out of 20), slide tracheoplasty (n=7; 35%, 7 out of 20), end-to-end anastomosis (n=5; 25%, 5 out of 20). Six patients (30%, 6 out of 20) had coexisting congenital intracardiac anomalies. There were 12 pulmonary artery sling (60%, 12 out of 20) patients. Overall operative mortality was 15% (n=3; 3 out of 20). Operative mortality was 20% (n=2; 2 out of 10) from 1986 to 2001 and decreased to 10% (n=1; 1 out of 10) from 2002 to 2011. All early deaths occurred in patients who had undergone patch tracheoplasty. Since 2004, there were no operative deaths. Seven patients (35%, 7 out of 20) required tracheal reintervention postoperatively. There were three late deaths (17.6%, 3 out of 17) at 8, 9 and 22 months after surgery. At last follow-up (mean 5.3±6.6 years; range 1 month to 18 years), all 14 survivors remained asymptomatic. Repair of tracheal stenosis in children has been associated with high morbidity and mortality. Since the introduction of slide tracheoplasty, a multidisciplinary team approach and abandonment of patch tracheoplasty, the mortality has been reduced. Survival beyond 2 years after surgery is associated with an excellent outcome.