Pulmonary surfactant is a lipid-protein complex that lines and stabilizes the respiratory interface in the alveoli, allowing for gas exchange during the breathing cycle. ATP-binding Cassette transporters A3, is a lipid transporter in the limiting membrane of lamellar bodies in alveolar type Ⅱ cells that plays a critical role in the regulation of pulmonary surfactant homeostasis.  Mutations in the ABCA3 gene cause respiratory distress syndrome in new-born and childhood interstitial lung disease.  An updated view on expression of ABCA3 gene and ABCA3 mutation associated with neonatal respiratory distress syndrome,or childhood interstitial lung disease was reviewed.