Melkersson-Rosenthal syndrome is characterized by a triad of signs: oedema of the face and lips, recurrent peripheral facial nerve paralysis, and fissured tongue (lingua plicata). Monosymptomatic or oligosymptomatic forms of Melkersson-Rosenthal syndrome are common. Aetiology is still unclear. Genetic, autoimmune and infectious factors have been proposed. The authors present a case of a 40-year-old man with Melkersson-Rosenthal syndrome treated with good effect by combination therapy consisting of intralesional injection of triamcinolone, and oral dapsone. The authors also review the literature of clinical presentation and current treatment of this rare syndrome.