ChILD secondary to surfactant defects is caused by mutations in: (i) SFTPB (MIM: 178640), and SFTPC (MIM: 610913), which encode surfactant protein components; (ii) ABCA3 (MIM: 610921), which encodes ATP-binding cassette sub-family A member 3, localized on the limiting membrane of LBs and essential for secretion; and (iii) NKX2.1 (MIM: 610978), which encodes a transcription factor regulating SFTPB, SFTPC and ABCA36,7,9. Here, SFTPB is linked to interstitial lung disease specific to childhood.