Congenital lobar emphysema (CLE) is a clinical and radiological entity with a multiplicity of etiological factors. Six patients are presented with CLE of the left upper lobe. In three of them CLE was associated with cardiovascular malformation i.e. persisting ductus arteriosus and in one case additionally with double aortic arch. These infants were treated by division of the compressing vascular structure without lobectomy. Only one required secondary resection. Left sided CLE presumably caused by vascular compression should be considered a special group in which surgical correction without lung resection is justified.