Twenty-one cases of congenital tracheal stenosis seen at the Royal Alexandra Hospital for Children 1971 through 1980 were reviewed with regard to the clinical features, associated anomalies, endoscopic findings and radiological evaluation. Congenital tracheal stenosis was usually a serious problem often associated with other major anomalies of the respiratory tract, esophagus, or skeleton. There was no set pattern of presentation. The presenting features included: persistent wheeze or stridor, atypical "respiratory distress," and atypical "croup" or "bronchiolitis." Documentation of the nature and extent of the stenosis by endoscopy and, if necessary, tracheobronchogram was important for prognosis and to direct treatment. Congenital tracheal stenosis must be considered in infants who present with atypical respiratory tract obstruction, especially where there is an H-type tracheoesophageal fistula, pulmonary hypoplasia or skeletal abnormalities such as hemivertebrae or a thumb abnormality.