Bronchial atresia with mucocele and focal hyperinflation of the lung is a rare anomaly. We report the observation of a 12-year-old girl which presented a right hilar opacity on chest X-ray. The thoracic computed tomography identified an atretic segmental bronchus of the middle lobe with mucocele and focal hyperinflation of the lung. Bronchial endoscopy ruled out any acquired etiology of bronchial obstruction. The definitive diagnosis was bronchial atresia with mucocele and focal hyperinflation of the lung. This uncommon malformation is usually a radiological description. Eighty-four cases were collected in the main series in 1989. Nowadays, about 12 new cases have been published.
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