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Granular cell tumors are relatively uncommon benign laryngeal lesions thought to originate from Schwann cells. The granular cell tumor occurs everywhere in the body, especially in the oral cavity. Most oral cases are located in the tongue. It has no preference for race, sex, and age. Because pseudoepitheliomatous hyperplasia is frequently associated with granular cell tumors, it should be differentiated from squamous cell tumors. Confirmative diagnosis should be made histopathologically and supported by immunohistochemical staining using S-100. Treatment of a granular cell tumor consists of a wide local excision by the endoscopic, transoral or laryngofissure methods. Recently, CO2 laser has been used to remove granular cell tumor with clear resection margin. We have recently experienced a granular cell tumor of larynx, so we report it with a review of literatures. (Korean J Otolaryngol 2002;45:1114-7)