The problem of congenital lobar emphysema remains relevant due to the complexity of differential diagnosis, possible long asymptomatic course, rapid deterioration of child condition and the need for decision to provide surgical treatment. Lobar, progressive emphysema of newborns (obstructive, intense, hypertrophic emphysema, etc.) – congenital defect; which is accompanied by a sharp progressive swelling of the lung particle. The clinical picture of congenital lobar emphysema varies widely from light pulmonary involvement to acute respiratory failure and pulmonary infections, most patients have no symptoms in the early postnatal period, most often they occur up to 6 months of age (in 50% of cases). The valve mechanism leads to the acute occurrence of bloating of the pulmonary parenchyma, an increase in the particle in the volume and compression of normally developed functioning pulmonary tissue, as well as the displacement of the mediastinum in the "healthy" side and acute violation of the blood circulation. Surgical intervention (lobectomy by thoracotomy and video-assisted lobectomy) is a curative method of choice. A very small proportion of patients had good results after conservative treatment, confirming the clinical case submitted for consideration. In child B., from birth to age of 7 months, no symptoms of respiratory failure, developmental disorders were not detected. After an acute respiratory viral infection there was an acute deterioration with a clinic of pulmonary heart failure, broncho-obstructive syndrome. Conservative treatment, the use of traditional and high-frequency lung ventilation were not effective. Improvement occurred only after surgical intervention – left lateral thoracotomy, upper lobectomy, sanation and drainage of left hemoutorax. In the postoperative period there were no complications. A child without signs of respiratory failure has been transferred to a somatic department.The case presented for consideration points to the need to focus attention on the differential diagnosis of congenital lung defects in children with broncho-obstructive syndrome.