Leiomyosarcoma of the trachea is a rare primary tumor of the upper airway. Twelve cases have been reported in the English-language literature. Our experience with a patient with leiomyosarcoma of the cervical trachea is presented. The classic symptoms, diagnosis, and surgical management of this case are contrasted with those of the previously reported cases. The surgical technique used for our patient, primary tumor resection with skin grafting and placement of a Montgomery Laryngeal Stent, is a unique and effective treatment.