The authors report the case of a 14-year-old girl with Melkersson-Rosenthal syndrome in its classical form, with the triad of recurrent peripheral facial paralysis, upper lip edema and fissured tongue. A rare disease of unknown etiology, it has a chronic and progressive course, and clinical diagnosis is usually by exclusion. An individualized, multidisciplinary approach is fundamental for the treatment of the symptoms and avoids complications and permanent sequelae. The therapeutic options are varied, though most commonly include corticosteroid therapy and nerve decompression surgery.