Congenital pulmonary airway malformation (CPAM) is one of the many causes of respiratory distress, generally occurring in about 7 % of infants in the early neonatal period. This article describes a clinical case of CPAM in a neonate who presented with signs of severe respiratory failure from the fi rst hours of life. The repeated occurrence of pneumothorax, development of pneumonia and hypoxicischemic damage of the central nervous system complicated the course of the disease in this child during the neonatal period. The clinical features of the course of the disease and their correlation with the results of laboratory and instrumental studies are analyzed. The results of dynamic radiography of the thoracic organs and spiral computed tomography (CT) of the lungs are presented. The presented clinical case shows that congenital lung pathology, namely malformation of the pulmonary airways, may be an unrecognized cause of respiratory distress in the neonatal period for a long time. The presented case report shows that congenital lung pathology, especially CPAM, in neonates may be an unrecognized cause of respiratory distress for a long time. In order to diagnose this disease promptly, a comprehensive evaluation of the child should include not only a chest x-ray, which is not always informative in the early stages, but also a CT scan of the lungs. Optimizing the choice of diagnostic measures contributes to increasing the eff ectiveness of treatment, including surgical treatment, and preventing the development of possible complications, improves the prognosis for life and health of children with the specifi ed pathology.