Congenital bronchoesophageal fistula associated esophageal atresia usually presents in the newborn period or infancy but those without esophageal atresia are more insidious in disease process. Symptoms which include cough, hemoptysis, choking on swallowing liquids, uncommonly dysphagia, and epigastric discomfort may not begin until adult life. Most of the cases are curative unless there are serious underlying conditions. The diagnosis is usually made by gastroesophagoscopy, esophagogram, bronchogram and bronchoscopy. And the most of the cases can be cured by fistulectomy and resection of involved pulmonary lobes. We experienced one case of congenital bronchoesophageal fistula which occurred in a 13- year-old girl who complained of paroxysmal cough and intermittent hematemesis for 3 years.