Melkersson-Rosenthal syndrome is a rare disorder characterized by a triad of recurrent orofacial swelling, recurrent paralysis of the facial nerve and lingua plicata. The complete triad only occurs in 25% of MRS cases. Monosymptomatic or oligosymptomatic variants are more frequent; other findings include granulomas in other facial sites, regional lymphadenopathy, fever, psychotic disorders and hyperplastic gingivitis are associated with MRS. This can be a diagnostic challenge. The etiology and pathogenesis of MRS are unknown. Corticosteroids or clofazimine appear the best therapeutic options.