In this case of bronchopulmonary foregut malformation (BPFM), the bronchus of the anomalous pulmonary tissue was reconstructed and tracheoplasty for associated congenital tracheal stenosis was performed at 5 days of age. After reconstruction the right lung was ventilated, which was followed by improved blood gas data. However, the persistent fatal circulation developed, and the patient died of respiratory insufficiency on postoperative day 5. The autopsy showed that both lungs were hypoplastic. This is the first case of bronchial reconstruction of BPFM.