Congenital Lobar Emphysema (CLE) is a rare malformation of lung development, which may be the cause of respiratory insufficiency in the suckling child. It is produced by the hyperinsufflation of one or more lung lobs with compression of the normal lung parenchyma and contralateral displacement of the mediastinum. Clinical diagnosis is confirmed by simple radiology exams. Lobectomy has better results than clinical treatment and should be chosen. Our purpose is to present one case of this rare illness in a newborn seen in our service with serious respiratory insufficiency and after complementary exams, CLE was confirmed. The patient was submitted to a surgical procedure (lobectomy) with an excellent post surgery evolution. Child is asymptomatic in long term follow up.