This report describes the first case of rhabdomyosarcoma of the trachea in a 65 year old man with a five week history of cough, wheezing, and hemoptysis. The tumor, presumably originating in the submucosal tissue, was characterized as an intratracheal polypoid mass showing no local invasion or distant metastasis. Rhabdomyosarcoma of the lower respiratory tract and its histogenesis are briefly reviewed and discussed.