Objective To explore the clinical characteristics and prognosis of congenital pulmonary artery sling (PAS) in children. Methods The clinical data of 38 children diagnosed with PAS during June 2009 and February 2015 were retrospectively analyzed. Results In 38 PAS children, 35 cases (89.47%) were hospitalized for varying degrees of respiratory manifestations with recurrent cough (89.47%) and wheezing (84.21%) being the most common. The remaining 3 cases were found abnormal in routine preoperative examination and the diagnosis was confirmed after further examination. All 38 children were performed computer tomography angiography (CTA). Thirty-seven cases were diagnosed of PAS and diagnostic rate was 97.37%. One case was suspected of pulmonary dysplasia and diagnosed of PAS after operation. Twenty-six children received surgical treatment, of whom 25 children had pulmonary artery reconstruction (LPA). Seven children died during/after operation and 18 survived. The remaining 12 children received non-surgical treatment, of whom 9 died and 3 survived. Conclusion Cardiac uhrasonography may reveal PAS in the early stage, while CTA is the best method for confirmed diagnosis. LPA reconstruction is an important means of relieving left pulmonary artery oppression.