Congenital lobar emphysema (CLE) is a rare malformation of lung development caused by the hyperinflation of the lung lobe with compression of the normal lung parenchyma and contralateral displacement of the mediastinum. It may represent immediately at birth with respiratory insufficiency or have a slower onset at 6 months of life. The treatment of choice may be conservative in mild cases or surgical removal of the affected lobe may be needed in severe cases that have persistent or progressive respiratory distress. A baby diagnosed as having CLE and whose affected lobe was surgically removed at the newborn period is presented and discussed.

Keywords: Congenital lobar emphysema, Lobectomy, Respiratory distress, Newborn