Background <p>Congenital bronchobiliary fistula (CBBF) is a developmental structural abnormality between the trachea or bronchus and the biliary tree, with non-specific clinical manifestations that mainly present as repeated cough and dyspnea, but it is often misdiagnosed as pneumonia. If not promptly diagnosed and effectively treated, CBBF can be fatal. Currently, radical surgery is the only treatment option for CBBF.</p> Case presentation <p>An 8-day-old full-term Han Chinese female infant presented with coughing accompanied by sputum and shortness of breath with worsening dyspnea for 4&#xa0;days. Yellowish green sputum was seen during invasive ventilation. A follow-up chest radiograph suggested a solid lesion in the right upper lung. Airway malformations and an abnormal opening in the middle of the tracheal eminence were observed by fiberoptic bronchoscopy. Chest computed tomography (CT) and tracheal imaging showed a fistula below the tracheal bulla traveling towards the liver and communicating with Glisson's capsule at the hepatic hilum, suggesting the presence of a tracheobiliary fistula. Intraoperatively, a fistula was seen extending from the tracheal eminence to the esophageal hiatus, and trans-fistula imaging showed that the contrast agent entered the intestines through the biliary tree, confirming the diagnosis of a CBBF, which was removed. The pathology was consistent with a bronchial fistula. The ventilator was withdrawn on postoperative day 10. Following discharge, the patient recovered well with good growth and development, and no symptoms of lung or digestive tract discomfort.</p> Conclusion <p>CBBF is frequently misdiagnosed. Bronchoscopy remains the preferred diagnostic modality for definitive confirmation.</p>