The congenital tracheoesophageal fistula without atresia of the esophagus is considered a rare variant, and the literature concerned to the tracheoesophageal fistula without atresia, is little in adult especially. The 22-year-old male was admitted to Yeungnam University Hospital with his chief complaints of weight loss (6kg/6 months), abdominal discomfort, and intermittent coughing. The diagnosis was made by the endoscopy and esophagography. The fistula was 1.5cm in diameter, 0.5cm in length. The level was around second thoracic vertebra. The operation was performed transpleurally through the right third intercostal space and the fistula was secured with interrupted silk suture after division. The fibrotic adhesion was seen around the tracheoesophageal fistula. The postoperative course was uneventful, and postoperative esophagogram revealed ho extraluminal leakage. Herewith we report this unusual case of isolated tracheo-esophageal fistula with review of literatures.