Granular cell tumors (GCT) are rare neoplasms and their diagnosis is mainly based on histopathologic examination of biopsies. Granular cell tumor is a neoplasm that has been reported in a variety of organs, including the extremities, head and neck skin, oral cavity and gastrointestinal tract. The cell of origin in GCT is controversial. In this article, we present a case of granular cell tumor of the tongue at the 49-year-old female patient with the knowledge of the literature.