Congenital subglottic stenosis of the larynx is one of the most common cause of chronic airway obstruction im infancy and childhood. It is defined as narrowing of the space bounded inferiorly by the inferior margin of the cricoid cartilage amd superiorly by the insertion of the fibers of the conus elasticus into the true vocal cords. In case we experienced was a female full-term baby delivered by Cesarean section. The stenosis was believed by hypertrophy of stromal soft tissue and cricoid cartilage in the subglottic area. The lesion was associated with tracheoesophageal fistula of H1 type. A brief review of the literature was done.