The case of a 59-year-old man with granulomatous glossitis is presented. The patient complained of increasing swelling of his tongue for years and a recent onset of perioral edema. Because of sarcoid-like granulomas found in a first lingual biopsy specimen, the disease was initially misdiagnosed as a localized form of sarcoidosis. A second biopsy specimen from the tongue revealed lymphonodular-plasmocytic granuloma-like infiltrates, which, in connection with the labial swelling, led to the diagnosis of Schuermann's glossitis granulomatosa as a peculiar manifestation of Melkersson-Rosenthal syndrome. The surface of the tongue showed deep furrows characteristic of lingua plicata. However, no history or signs of facial or trigeminal nerve paralysis were present; thus the patient did not show the complete triad of symptoms typical of Melkersson-Rosenthal syndrome. After treatment with clofazimine the perioral and lingual swelling disappeared within two weeks. Recurrences have been suppressed with a reduced maintenance dosage of this agent.