Congenital lobar emphysema is frequently a life-threatening disorder presenting in infancy. The diagnosis should be considered when the patient shows signs of respiratory distress and the x-ray demonstrates the characteristic hyperlucent lobe with compression of the surrounding lung tissue. The morphologic aspects and pathogenesis are not clearly defined, although it is thought that an abnormality of bronchial cartilage is probably associated with the development of lobar emphysema. The results of treatment by lobectomy are excellent, and the mortality with surgery is low. Consideration of nonsurgical management is worthwhile in mildly affected or asymptomatic patients. Long-term follow-up of both groups indicates a very favorable prognosis in this disease entity.