A seropositive patient who developed a granular cell tumor on the dorsum of the tongue is referred. The lesion was surgically excised. His clinical state is thoroughly studied, along with the histopathological and immunohistochemical examinations findings. Numerous histogenesis theories and the appropriate tumor treatment are mentioned within the article being always in accordance with the relative literature. The case mentioned contributes to the literature by being the first report of a seropositive patient with a tongue granular cell tumor ever noted.