A 72-year-old woman, who had sometimes coughed at meals for 2-3 years, was seen at the hospital because of recent aggravation of coughing at swallowing and epigastric pain. Upper gastrointestinal series indicated a diverticulum of the esophagus and fistula from the esophageal diverticulum to bronchus. Endoscopic examination of the esophagus confirmed the diverticulum in the middle portion of esophagus, and in its center the fistula was observed. Chest CT visualized that the fistula communicated with the right S6. A diagnosis of esophagotracheal fistula with esophageal diverticulum was made. At operation the pleural space was opened through the seat of the right-sided 5th lib, and the esophageal diverticulum and fistulous duct communicating with the peripheral bronchus were confirmed. Inflammatory findings were lacked in the diverticulum and around the fistula, so easy freeing was possible. Since no lesion was detected in the right lower lobe of the lung, excision of the esophageal diverticulum and fistulectomy were performed. Histopathologically there were stratified squamous epithelia covering from the esophageal diverticulum to the fistula duct. From the above the patient was diagnosed as adult congenital esophagotracheal fistula (Braimbridge I type). This disease is a relatively rare entity. This paper describes the case as well as other 54 domestic cases.