Melkersson-Rosenthal syndrome is an uncommon disorder of uncertain aetiology characterized by orofacial oedema, facial nerve palsy and lingua plicata. The triad is seldom seen in its complete form, and oligo-symptomatic or mono-symptomatic forms are more common. An unusual case of Melkersson-Rosenthal syndrome involving the left upper eyelid is presented. The pathology, clinical features and management of this disorder are discussed.