Aims & Objectives: Bronchobiliary fistula is a rare developmental abnormality. Until 2019 only 44 cases had been reported worldwide. Clinicians should have a high index of suspicion to make an early diagnosis and start prompt treatment to reduce mortality. All cases should undergo surgery in order to avoid pulmonary complications. Methods: We report a case of a 45-day-old female patient admitted to the Pediatric Intensive Care Unit after a Brief Resolved Unexplained Event. Severe gastroesophageal reflux was suspected but failed to conventional treatment and developed respiratory distress requiring mechanical ventilation Results: Bile was obtained through the orotracheal tube and a 3D computed tomography and bronchoscopy were performed to demonstrate anatomic malformation between the biliary tract and the bronchial tree. A bronchobiliary fistula was observed between the main right bronchus and the biliary tract. Surgery was performed on day 95 after birth requiring mechanical ventilation for 75 days. Pulmonary chronic disease was diagnosed at time of discharge and inhaled steroid was started immediately. Conclusions: Congenital bronchobiliary fistula is a rare disease; survival rates and prognosis depends on a high index of suspicion to make an early diagnosis and rule out other biliary tract malformations. Prognosis rates are improved managing pulmonary complications due to biliary fluid in the pulmonary tree until a surgical treatment can be carried out.