An infant with congenital left upper lobe emphysema presenting with respiratory distress and failure to thrive, which necessitated lobectomy, is described. Careful pathological examination revealed two defects in association with the emphysema: bronchial atresia and defective bronchial cartilage in the affected lobe. Whilst congenital lobar emphysema is the term usually employed to describe this clinical presentation of respiratory distress in infancy, 'panlobular emphysema of infancy' would be a more accurate description. Congenital defects in the airways may account for the development of emphysema, but there may be a more fundamental congenital tissue abnormality at alveolar level.