RESUMEN
The aim of the study is to share a series of cases presented with respiratory distress in neonatal period or infancy due to uncommon case of congenital lobar emphysema. Different modes of presentation and diagnostic work up is discussed. Congenital lobar emphysema results from hyperinflation of a segment of lung from incomplete obstruction of major airway. This result in respiratory distress due to compression of other lobe of same lung and contra lateral lung. A series of neonates and infants under 2 years of age presented with respiratory distress due to congenital lobar emphysema at Liaquat National Hospital,Karachi. These children presented with progressive respiratory distress and underwent thoracotomy and lobectomy. In most of the cases, diagnosis was obvious on plain chest X-ray, but to confirm, CT scan and/or perfusion radioisotope scan is performed. Thoracotomy and lobectomy gave excellent results