Congenital lobar emphysema is one of the abnormal development of pulmonary parenchyme which may cause respiratory distress in infant. This disease is an unusual condition barely mentioned before 1949, and only 166 cases were reported till 1967 in English literature. This report describes a 13 months old girl who had a congenital left upper lobar emphysema. The left upper lobectomy was the operation for this case and the postoperative course was not eventful, and have been in good condition upto now.