Abstract
Congenital lobar emphysema (CLE) is a rare developmental malformation that presents as neonatal respiratory distress and can be successfully managed with prompt intervention. Hyperinflation of the affected lobe with mediastinal shift is the characteristic radiological finding. However, the etiology mostly remains unknown. Here, we report a case of CLE that microscopically revealed bronchial cartilaginous hypoplasia as an underlying cause of this malformation.