A 5 mo old male intact English bulldog was evaluated at a veterinary referral hospital for acute respiratory distress and chronic difficulty breathing. Thoracic radiographs revealed multifocal pulmonary hyperinflation and hyperlucency suspected in the left caudal and accessory lung lobes. A thoracic computed tomography scan identified severe diffuse enlargement of the caudal subsegment of the left cranial lung lobe and the dorsal process of the accessory lung lobe, with parenchymal hypoattenuation, rounded margins, and thin pulmonary vessels. Based on clinical signs and imaging findings, he was diagnosed with suspect congenital lobar emphysema in multiple lung lobes. A median sternotomy was performed, which revealed a hyperinflated, emphysematous left cranial lung lobe (caudal subsegment) and accessory lung lobe for which two lung lobectomies were performed. The remaining lung lobes were small and atelectatic. Histopathology revealed bronchial cartilage hypoplasia and aplasia and findings consistent with congenital lobar emphysema. The puppy recovered well from surgical treatment of congenital lobar emphysema, requiring multiple lung lobectomies, with subsequent computed tomography–evidenced re-expansion of the remaining lung lobes 3 mo after surgery. The patient is still alive 1 yr after surgery with a normal activity level and no evidence of respiratory compromise.