In most cases, SFTPB deficiency is associated with congenital alveolar proteinosis with an accumulation of granular, eosinophilic, PAS-positive, lipo-proteinaceous material in the alveolar spaces and frequent desquamated AEC II and foamy macrophages; the aspect of desquamative interstitial pneumonitis is less frequently seen [1]. This evidence concerns the gene SFTPB and Intraalveolar phospholipid accumulation.