Granular cell tumor is a relatively uncommon benign hamartomatous lesion occurring in almost any part of the body. The tongue and the buccal mucosa are common intra oral sites. Granular cell lesions may be found in other diverse sites such as the jaw, skin, gastro intestinal tract and respiratory tract. The histogenesis of the lesion still remains unknown. However, histochemical and ultra structural studies propose the origin of the lesion from schwann cells, striated muscle, mesenchymal cells, histiocytes and epithelial cells. The tumor generally occurs in middle or older aged adults. The lesion is typically seen as an uninflammed asymptomatic mass measuring about two cms in diameter with a yellowish surface coloration. As most of the granular cell tumors are benign, surgical excision of the lesion is the treatment of choice. We describe a case of granular cell tumor of the tongue in a 6 year old girl along with a brief review of literature on granular cell tumors.