Severe congenital stenosis of the entire trachea with absence of the membranous portion caused acute respiratory obstruction in a 4½-year-old boy and required emergency tracheal replacement. The surgical repair consisted of a unique in situ esophageal replacement of the trachea and tracheo-esophageal anastomosis with an internal stent. The replacement worked well for 39 days at which time the patient died suddenly from a plug of mucus in the trachea, which occluded the anastomosis. Further experience with tracheal replacement and use of in situ esophagus for long tracheal strictures appears justified.