Pulmonary artery sling (PAS) is a rare congenital cardiovascular disease and usually associated with airway and cardiovascular anomalies. Infants may present with recurrent stridor, infections and dyspnea, and older children may present with dysphagia. Asymptomatic cases may also be found. PAS is easy to be misdiagnosed as other breathing disorders. Imaging examination plays an important role in the diagnosis of PAS. Diagnosis mainly depends on finding relationships between the abnormal left pulmonary artery and the airway by CT and three-dimensional reconstruction. Early diagnosis and treatment is the key point. The traditional treatment for PAS is the left pulmonary artery reconstruction and narrow tracheoplasty. It is still controversial in the management of PAS associated with trachea stenosis. This article reviews the recent progress of definition, classification, clinical manifestation, auxiliary examination, diagnosis, and management of PAS.