The clinical course of our patient (early onset and unexplained nRDS in a term infant, evolving to severe respiratory failure despite maximal respiratory support, with lung histology) marked by AEC II hyperplasia, numerous macrophages in the alveolar lumen, focal alveolar proteinosis, extended areas of desquamative interstitial pneumonia, and the results of SFTPB sequencing suggests a possible surfactant metabolism defect due to SFTPB deficiency. This evidence concerns the gene SFTPB and respiratory failure.