Leiomyosarcomas account for approximately 7% of all soft-tissue sarcomas and occur most frequently in the gastrointestinal tract and uterus. Leiomyosarcomas of the larynx are extremely rare with only about 27 cases reported in the available literature. We present a leiomyosarcoma of the left vocal cord that was treated by transoral CO2 laser surgery. To date, the patient has been free of disease for more than 25 months. Since these tumors may be difficult to diagnose, immunohistochemical studies may be necessary for making an unambiguous diagnosis. Primary treatment should be surgical resection, with small tumors suitable for partial laryngectomy or transoral laser surgery.