[An unusual cause of respiratory failure. Bronchobiliary fistula]

The authors describe the treatment of a patient with an inborn bronchobiliary fistula. The ex post finding that the fistula had two insertions in the bronchus called in the final stage for a combined thoraco-abdominal approach with resection of the right lower pulmonary lobe with ligature of the fistula beneath the diaphragm. Although inborn bronchobiliary fistulae are very rare, they must be considered in the differential diagnosis of causes of chronic respiratory distress syndrome in children. Early diagnosis and adequate surgical treatment make it possible to prevent serious chronic cardiorespiratory disease.