Congenital stenosis of the trachea (CST) is one of the most unusual anomalies of the respiratory tract. Recentry we experienced a case of CST associated with multiple malfor-mations. Among the reported cases up to the present, this is the first case who has associated with gastrointestinal malformations such as Hirschsprung's disease and malrotation. The patient was surgically treated for these alimentary tract anomalies except that of trachea by the 20th day after birth. However her postoperative course became worse and she died of respiratory distress on the 5th postoperative day. An autopsy reveald a full-length tracheal stenosis due to encircled cartilagenous ring.
