ATP binding cassette transporter A3（ABCA3）is a critical protein involved in phospholipid metabolism in typeⅡ alveolar cells，participating in the synthesis of pulmonary surfactant.Early studies have found that mutations in ABCA3 gene can lead to childhood interstitial lung disease（chILD），but the underlying mechanisms remain unclear.Recent elucidation of the ABCA3 structure，coupled with functional inquiries into the protein，has engendered fresh insights into the intricate mechanisms governing phospholipid metabolism orchestrated by ABCA3，inspiring the development of small molecule drugs targeting ABCA3 gene mutations.This article provides a comprehensive review of the involvement of ABCA3 in phospholipid metabolism，the pathogenic mechanisms of related lung diseases，the genotype-phenotype correlations，and the forefront advances in treatment.Additionally，it underscores lingering unresolved queries，aiming to provide a platform for the future refinement of precision treatments for ABCA3 mutations.