Rhabdomyomas are benign tumors of skeletal muscle derivation. Rhabdomyomas of the head and neck are unusual, and laryngeal rhabdomyomas of the adult type are rare. When they do occur, patients may present with symptoms that have progressed slowly over several years. In extraordinary cases, stridor and airway obstruction manifest. We report a case of adult rhabdomyoma of the larynx that features some of its typical and atypical presenting characteristics. This case also exemplifies the favorable prognosis that results following complete yet conservative surgical excision.