Background: congenital lobar emphysema is a rare cause of respiratory distress in neonates and infants. It was treated by lobectomy since 1943. Objectives: The aim of this study was to present our experience in the diagnosis and surgical treatment of congenital lobar emphysema cases and highlight the effect of multidisplinary team work on the outcome. Methods: We retrospectively analyzed data of patients operated for congenital lobar emphysema at cardiothoracic surgery department, Sohag university hospital over the period of 15 years starting from January 2000 and ending in December 2015. Results: Total number of cases was fifty three. Males were 39 (74%) while females were 14 (26%). mean age was 12 weeks. Six patients (11%) had associated cardiac anomalies. main clinical presentations were acute respiratory distress and recurrent chest infection. Left upper lobe affected in 35 cases (66%), right upper lobe in 10 cases (19%) and right middle lobe in 8 cases (15%). all cases subjected to anatomical lobectomy by thoracotomy . One patient subjected to patent ductus arteriosus ligation simultaneously with Left upper lobectomy at the same operative setting. Post operative complications reported in 8 cases (15%) and one patient of them died from sepsis and respiratory failure (1.9%). Postoperatively, all surviving patients were followed up for one year in the outpatient clinic. Conclusion: congenital lobar emphysema is a life
threatening disease. Early diagnosis and surgical management by amulti-displinary team is the golden key for favorable and safe outcome.