Introduction. Interstitial lung diseases in newborns associated with diffuse pulmonary developmental disorders are a difficult to detect, as they are rare, formed at the early stages of embryonic development, clinically manifested in the first hours of life as severe respiratory distress syndrome in newborns. The aim. To demonstrate the role of radiation research methods and the difficulties of differential diagnosis of rare forms of interstitial lung diseases associated with diffuse impaired lung development. Materials and methods. Clinical cases of congenital alveolar dysplasia and alveolar-capillary dysplasia with an abnormal location of the pulmonary veins are described in detail, the radiological data, confirmed by the histological data, are presented. Results. Specific changes in the lungs in ILD of newborns associated with a diffuse disorder of lung development, according to X-ray data in the first day of life, are not determined, however, a progressive negative trend has been noted. The lungs computed tomography allows a detailed diagnosis of structural changes in the lung parenchyma, their severity and prevalence. Issues of differential diagnosis of diffuse disorders of lung development in newborns are discussed; the obtained results correlate with the published data. Conclusions. Histological examination is the “gold standard” in the diagnosis of interstitial lung diseases associated with diffuse lung developmental disorders in newborns, but is most often performed at autopsy. The role of radiological methods in the algorithmic approach of diagnostics is increasing due to the accumulated data, the improvement in the quality of detection and recognition of rare variants of interstitial lung diseases in newborns according to CT of the chest organs.