Leiomyosarcoma of the larynx is an extremely rare entity. This tumor is aggressive and is associated with dismal treatment results. We present a case of leiomyosarcoma of the larynx treated with surgery and postoperative radiotherapy. In this article we also discuss laryngeal leiomyosarcoma's clinical manifestations, associated diagnostic dilemmas, and optimal management, as well as a review of the literature.