Melkersson-Rosenthal syndrome (MRS) is a rare neuro-mucocutaneous syndrome of unknown etiology with chronic and progressive course, and clinical diagnosis is usually by exclusion. Rarely, it presents with the full triad of recurrent or persistent facial swelling, relapsing facial palsy, and a fissured, geographic tongue. It is rarely possible to observe all aspects of the classical triad at the same time, since these symptoms may appear in different times of life cycle. Although etiology of MRS is unclear, various factors such as infections, genetic predisposition, immune deficiency, food intolerance and stress have been held responsible. The differential diagnosis of MRS includes also chronic inflammatory and infective diseases characterized by granulomatous infiltration, as well as rosacea, contact dermatitis, allergic reactions and Bell’s palsy. The therapeutic options are varied, though most commonly include corticosteroid therapy and nerve decompression surgery