Background : Congenital bronchoesophageal fistula(BEF) presented in adult life is a rare disorder and has characteristic clinical findings such as paroxysmal cough after water ingestion and recurrent respiratory infections. It usually manifested recurrent pneumonia and chronic cough with purulent phlegmon which was mis-or under-diagnosed as chronic bronchitis, bronchiectasis or lung abscess so forth. Methods : We reviewed retrospectively 13 cases of congenital BEF in adult of Paik Hospital, College of Medicine, Inje University including 22 cases of congenital BEF previously reported in literature of Korea from 1979 through 1995. Results : The mean age at diagnosis was . There was no difference in sex ratio(Male : Female 18 : 17). The most common symptom was cough(91.4%), followed by chronic sputum(74.3), hemoptysis(25.7), and paroxysmal nocturnal cough at specific position(20%). Twenty one of 31 patients who were able to review have the most specific sign, Ono's sign presented as paroxysmal cough after liquid ingestion. By classification of Braimbridge-Keith, Fourteen(45.1%) of 31 patients were group I (associated with esophageal diverticulum), 15(48.4%) were group II (simple fistula), and group Ill and IV was one case in each. The opening of fistula confined to right lower lobe in 26(76.5%), left lower lobe in 6(17.6%), and left main bronchus in 2(5.9%) cases. Conclusion : Congenital bronchoesophageal fistula is uncommon disorder which has characteristic histories and specific symptoms such as chronic and recurrent lower respiratory infections, and paroxysmal cough after liquid ingestion. Medical attention and careful history should be done in patients who have localized recurrent lower respiratory infections in right lower lobe.