Introduction: Disorders of the surfactant are a rare category of chILD [Interstitial Lung diseases in childhood]. Their medical awareness is low leading to underreporting, delayed diagnosis and increased costs to patients.

Aims and objectives: To systematically review the literature and report their demographic, clinical and imaging features, possible treatments and prognosis.

Patients and Methods: PubMed was searched for relevant case reports, case series, and observational studies related to surfactant -related chILD up until April 2023. Data were extracted and analyzed accordingly.

Results: 137 eligible articles were included and 261 cases were analyzed. The main affected genes were SFPTB, SFPTC, ABCA3 and NKX 2-1. The median age of onset was 4 months (Q1:0/Q3:18) and the median age of diagnosis 14 months (Q1:3/Q3:79,5). The most frequent symptoms were dyspnea/tachypnea (98,3%) and respiratory failure (95,3%). The main radiologic finding were diffuse bilateral ground glass opacities (92,8%). The SFPTB, homozygosity, positive family history and earlier age of onset were associated with worst prognosis (p-value: <0,001, 0,036, 0,022 and 0,004 respectively). Treatment with corticosteroids, hydroxychloroquine and azithromycin was related to lower mortality (p-value:  0,021, <0,001 and 0,003 respectively). The 14 observational studies reported mixed results regarding treatments and outcomes.

Conclusions:  Due to the rarity of the disease, the literature is of low methodologically quality, comprising mostly of case reports/series and small observational studies. Larger and more robust studies need to be conducted, especially clinical trials investigating different treatment regimes.