Granular cell tumor, a relative rare neoplasm, is of particular interest of the otolaryngologist, as some 50% of cases appear in the head and neck region. Individual tumors are usually small, nodular, and nonulcerating. There appears to be no predilection for sex, and most tumors occur between the third and sixth decades of life. Pseudoepitheliomatous hyperplasia is frequently associated with granular cell tumor. Wide local excision, the treatment of choice, should be curative. Despite the usually benign nature of granular cell tumor, its propensity for local invasion, multicentricity, and associated pseudoepitheliomatous hyperplasia should alert surgeons to its potential hazards, to the probability of recurrence if it is only marginally excised, and to the possible later development of similar lesions. The authors experienced a case of granular cell tumor of the larynx which was comfirmed by histopathologic study.