Rhabdomyosarcoma of the larynx is extremely rare in adults, as only 17 well-documented cases have been previously reported in the English-language literature. Of these, only 2 cases (both male) involved the alveolar subtype of rhabdomyosarcoma, and neither involved the true vocal folds. We report a case of alveolar rhabdomyosarcoma of the true vocal fold in 54-year-old woman. She was successfully treated with conservative surgery, radiation, and chemotherapy. Management of head and neck rhabdomyosarcoma has evolved from radical surgery to less morbid procedures supplemented with radiation and chemotherapy. However, because this tumor is so rare in the adult population, no adult-specific treatment regimen has emerged. Nevertheless, the success of treatment in the pediatric population supports its use in adults.