Pulmonary Artery Sling(PAS) is a rare congenital vascular disease, which complied with airway stenosis, bridging bronchus, congenital heart disease and congenital anal atresia. As we known, it sometimes combined with several complex malformations. Children with PAS, especially with special facial features and other system malformation, should be alerted that the genes-chromosome lesions happened. We reviewed PAS for the definition, pathological mechanism, diagnosis and therapy in this article.