Granular cell tumor of the tongue is a relatively uncommon benign lesion of unknown etiology, uncertain histogenesis and nature. It may be single, occasionally multiple, and rarely becomes malignant change. The lesions occur in various part of the body, but the oral cavity, particularly the tongue, appears to be the most common location. It commonly revealed as an isolated or infiltrating solitary asymptomatic nodule, rarely over 3 cm in diameter, involving subcutaneous or submucosal tissues. It is best treated by adequate local excision without aggressive management. Radiation and chemotherapy have no place in the treatment. The authors experienced a granular cell tumor of the tongue which was confirmed by histopathologic study.