Background and Objectives Airway compression (AC) often complicates the perioperative course of congenital heart disease repair. Recent computed tomography (CT) helps the accurate diagnosis of this condition and the additional surgical procedure to relieve AC has been performed increasingly. The aim of this study is to identify the risky conditions of AC and to document the late outcome of the children who had airway narrowing associated with congenital heart disease. Subjects and Methods The medical records of 71 patients who were diagnosed as congenital heart disease with AC on cardiac CT between January 1999 and December 2007 were reviewed retrospectively. The duration of follow up was 52.27짹29.39 months. Results Median age of AC diagnosis was 5 (0-105) months. The AC lesions were most commonly observed in the left main bronchus (62%), followed by the trachea (18.3%) and both main bronchus (11.3%). Of total 71 patients, 33 patients had AC preoperatively; vascular ring, coarctaction of aorta, and absent pulmonary valve syndrome were common diseases. In 2 patients surgical procedures for AC relief were performed at initial operation. In the 38 patients, AC was found after initial operation; aortic arch repair for coarctation or interruption (44.7 %) and infantile Rastelli operation (18.4 %) were highly associated procedures. Seven patients (9.9%) required subsequent secondary surgery (aortopexy) for AC at the age of 11.4짹9.8 months. After aortopexy, airway compression was improved in 4 patients (57 %). During the follow-up period, 26.8% had a history of readmission because of respiratory infection. Only 1 patient who had pulmonary artery sling and tracheal stenosis died of airway problem. Four patients died of other causes. Currently most patients who didn���t undergo specific AC surgery (76%) shows no symptoms associated with airway problem in average 4.3 years of follow-up. Conclusion Children with congenital heart disease are at risk of airway problem both before and after cardiac surgery. Although chronic airway compression in infancy carries a significant morbidity, most of patients who had airway problem became asymptomatic after several years.