Congenital bronchial stenosis is a very uncommon condition in children. A full-term neonate showed severe respiratory distress soon after birth. Bronchoscopy and spiral computed tomography with multiplanar reconstruction disclosed a short stenosis of the left main bronchus, next to the carina, and another stenosis in the intermediate bronchus with air trapping in the right middle and lower lobes. The child underwent resection and reconstruction of the left main bronchus, and right middle and lower lobectomies after a failed attempt of bronchoscopic dilatation of the intermediate bronchus. A temporary silicon tracheal stent was necessary to achieve permanent extubation. The patient was discharged in good condition without any oxygen dependency and remains asymptomatic 1 year after surgery. Management of children with airway stenosis is complex, and surgeons involved in it should be familiar with multiple surgical and endoscopic techniques. A team approach in a referral center is the best option to achieve an optimal result.
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