Drugs capable of increasing the functionality or expression of the mutated proteins (i.e., ABCA3), antifibrotic drugs (already used in adult patients with pulmonary interstitial disease), and, above all, gene therapy (using viral vectors in the case of surfactant protein variants) may allow the development of targeted treatments for congenital surfactant protein deficiency. This evidence concerns the gene ABCA3 and protein S deficiency.