Congenital lobar emphysema is a very rare congenital cystic malformation of the lung that can cause acute respiratory distress early in life. This study reviews 19 cases of CLE over a period of 5 years at a Tertiary Pediatric Referral Center. The cases were studied with special emphasis on Histopathological findings of the lobectomy specimens, particularly bronchial cartilage abnormalties, radial alveolar count and their predictive value in pathogenesis of CLE. This condition can mimick other causes of respiratory distress in the neonatal period. Early diagnosis and effective surgical management is curatve.