A 2 mo old domestic shorthair kitten was presented for acute respiratory distress and severe ambulatory difficulties. Thoracic radiography revealed hyperinflation of the left cranial lung lobe and a mass with soft-tissue/gas opacity in the caudal mediastinum, leading to the suspicion of congenital lung lobe emphysema and hiatal hernia. Decreased bone radiopacity and suspected pathological fractures were also present. Complete clinicopathological analyses showed significant ionized hypocalcemia and suspicion of secondary hyperparathyroidism related to an inadequate diet. Lung lobectomy and reduction of the hiatal hernia following a median sternotomy and a cranial laparotomy were performed. IV and oral supplementation of calcium led to a full recovery and improvement in the kitten’s walking. A histopathological analysis revealed pulmonary emphysema associated with hypoplastic and irregular bronchial cartilage. Congenital lobar emphysema is a rare disease in both humans and animals. This is the first veterinary report describing a kitten affected by congenital lobar emphysema combined with a hiatal hernia and additionally complicated by secondary nutritional hyperparathyroidism with a good long-term outcome.