Congenital lobar emphysema is a rare variety of congenital malformation of lung characterized by over distension of a lobe of a lung due to partial obstruction of the bronchus. We are reporting a neonate admitted in the pediatric emergency ward with the respiratory distress since 16th day of life.Investigation revealed the overexpansion of the left upper lobe with mediastinal herniation, shifting of the mediastinum to the opposite side and collapse of the ipsilateral lower lobe. The baby was treated with conservative treatment and the condition of the baby was improved.