Congenital pneumonia is one of the main causes of high neonatal morbidity and mortality. The incidence of congenital pneumonia is about 1% among full-term infants and about 10% in premature infants and it is the cause of 10–38% of neonatal deaths. The etiological factor of this nosological entity can be bacteria, viruses, protozoa and fungi. The onset of clinical manifestations of congenital pneumonia depends on the period of action of the infectious agent (in utero, intranatally), as well as on the taxonomic characteristics of the pathogens. The lack of anti-infective protection in premature newborns, especially extremely and very premature ones, allows us to consider them as a specific group of immunocompromised patients, in whom congenital pneumonias occur more often and run a more severe course. In comparison with full-term babies, premature ones have some pathogens (ureaplasma, mycoplasma) that are often associated with the development of bronchopulmonary dysplasia in the outcome of congenital pneumonia. In addition, congenital infections caused by the same pathogen in premature infants can run a fulminant course, be accompanied by septic shock, respiratory distress syndrome, and death occurring within a few hours. This review of the literature presents an update on the etiology and pathogenesis of congenital pneumonia in newborn infants. Understanding the presented links of pathogenesis will be able to timely and correctly perform treatment in such babies.