Published reports of benign nerve sheath tumors of the larynx are extremely rare. Because these tumors represent a potential threat to the airway, prompt diagnosis and treatment are vital. We report a case of a neurilemmoma that arose from the right arytenoid muscle adjacent to the arytenoid cartilage. The mass was completely excised, and no recurrence was observed during follow-up. We discuss the diagnosis and management of this rare tumor.