Abstract: Objective To evaluate the difference of tracheobronchial stenosis and airway malformation between double aortic arches and pulmonary artery sling in children. Methods Clinical feature and imaging data of spiral CT were retrospectively analyzed in children with double aortic arches or pulmonary artery sling who was hospitalized from July 2010 to July 2015. Results There were 16 children (11 males and 5 females) with double aortic arches whose median age at onset was 3.5 months old. There were 47 children (28 males and 19 females) with pulmonary artery sling whose median age at onset was 4-month-old. In these 16 cases of double aortic arches, 14 cases were coupled with tracheobronchial stenosis. Two cases had more than one segment involved and they were segments II or III. Twelve cases had only one segment involved. Different degrees of tracheobronchial stenosis occurred in 47 cases of pulmonary artery sling, among whom 27 cases had more than one segment involved and the common segment was II and III (19 cases). Two cases had four segments involved and 20 cases had only one segment involved. There was statistical significance in the number of involved segments between children with double aortic arches and pulmonary artery sling (χ2=13.588, P=0.001). In 16 cases of double aortic arches, one case was combined with tracheal bronchus, and 1 case was combined with pulmonary hypoplasia. In 47 cases of pulmonary artery sling, 8 cases were combined with bridging bronchus, 3 cases combined with tracheal bronchus, 3 cases combined with pulmonary hypoplasia, 5 cases combined with bronchial deficiency or tracheal diverticula, and 2 cases combined with bronchopulmonary foregut malformation. There was statistical significance in the number of cases combined with airway malformation between children with double aortic arches or pulmonary artery sling (χ2=5.333, P=0.021). Conclusions The tracheobronchial stenosis and pulmonary airway abnormalities are more prominent in children with pulmonary artery sling than those in children with double aortic arch.