Background: Congenital bronchobiliary fistula (CBBF) is a rare disease with abnormal connection between the biliary tract and the bronchus. The misdiagnosis rate of CBBF is high, delayed surgery may induce poor outcome. Case presentation: We reported a girl with CBBF who was confirmed via bronchoscopy and chest computed tomography (CT). The girl was 7-month-old admitted to the hospital with “recurrent pneumonia”. Bilirubin crystallization detection was positive in the bronchoalveolar lavage fluid (BALF). CT images showed the abnormal traffic between the biliary tract and the right main bronchus, the girl was diagnosed with CBBF and recovered after surgical operation. Literature search: A total of 27 cases were collected, 14 (51.8%) cases were diagnosed in the neonatal period, 9 (33.3%) patients were diagnosed in infancy. CBBF was more common in female (19/27, 70.3%). The specific manifestations were bilious sputum (24/27, 88.9%) and bilious vomiting (7/27, 22.2%). Recurrent pneumonia (9/27, 33.3%) was common clinical manifestations. The most abnormal fistula originates different location around the carina was right main bronchus (21/27, 77.8%). There were 23(85.2%) cases recovered after surgical operation and 4(14.8%) cases with severe comorbidities/complications died. Conclusions: CBBF should be suspected in infants with bilious sputum. Bronchoscopy plays crucial role in preoperative evaluation and postoperative follow-up. With early diagnosis and surgery, the prognosis is quite well for CBBF patients without severe biliary malformation and/or severe postoperative complications.