A 50-year-old man was admitted to our clinic with a complaint of lingual enlargement. Detection of non-caseous epithelioid granuloma on histopathological examination led to a diagnosis of a granulomatous glossitis. Extensive investigation for the presence of associated disorders yielded negative results. Metranidazole and clofazimine were totally ineffective and tetracycline led to a minimal improvement. No associated disorder was detected at a 4-year follow-up examination. The position of granulomatous glossitis within the spectrum of orofacial granulomatous conditions is discussed.