Background: Congenital lobar emphysema evolution and morphofunctional changes of the lung parenchyma is an actual problem for the clinicians. Material and methods: The study has analyzed a medical history, clinical and imaging data and, particularly, the results of the histopathological(lung tissue samples) and histobacterioscopic examinations of 11 patients aged 0-6 months with congenital lobar emphysema (CLE) that have undergone a surgical treatment. Among them the newborns have constituted 54.5% (6 cases), the children aged 2-3 months – 36.4% (4 cases). The study has stated that CLE pathogenesis involves a large number of congenital, nonchromosomal pathologic processes that represents the ontogenesis abnormalities in embryogenesis, fetal development and neonatal periods. Results: In terms of histopathology, these processes can be classified into 3 groups: dysplastic changes, inflammatory changes and tissue immaturity (dysmaturity). This classification contributes to the development of a new histopathologic concept of ethiopathogenetic forms of CLE. Although the inflammatory changes have statistically predominated in all tissue sample groups, the inflammation has not been a determinant but a secondary factor in the development of dysplastic changes or immaturity, what essentially complicates the assessment of preexistent congenital abnormalities and the disease evolution, especially, in the neonatal period. Conclusion: The histobacterioscopy of tissue samples with inflammatory changes has not detected any microorganisms. The authors suggest that a surgical intervention (lobectomy) immediately after the diagnostication is the optimal treatment as it provides good results in the short, middle and long terms.