Melkersson-Rosenthal syndrome(MRS) is a rare, noncaseating granulomatous disorder of unknown etiology and undefined diagnostic criteria. The classical triad of recurrent orofacial edema, relapsing facial paralysis, and fissured tongue, is not frequently seen in its complete form, and many patients remain misdiagnosed or undiagnosed for years. The purpose of this study is to review the findings in the literature describing theMelkersson-Rosenthal syndrome with aim to identify its clinical and histopathological characteristics and correlate them with definitive diagnostic criteria and effective treatment modalities. A systematic review and analysis of more than 100 publications met eligibility criteria performed by the authors. Orofacial edema of unknown etiology is the most typical clinical feature of the Melkersson-Rosenthal syndrome. Its coexistence with of facial nerve palsy or fissured tongue could be characterized as an oligosymptomatic MRS. Many investigators suggest cheilitis granulomatosa as a monosymptomatic form of MRS, while patients with facial palsy and fissured tongue, without orofacial edema, should not be considered having MRS.Histological evidence is not necessary. Corticosteroids are generally accepted as the mainstay treatment. The classical triad of recurrent orofacial edema, relapsing facial paralysis, and fissured tongue, is not frequently seen in its complete form, and many patients remain misdiagnosed or undiagnosed for years. The purpose of this study is to review the findings in the Literature describes the Melkersson-Rosenthal syndrome with aim to identify its clinical and histopathological characteristics and correlate them with definitive diagnostic criteria and effective treatment modalities. A systematic review and analysis of more than 100 publications eligibility criteria performed by the authors. Orofacial edema of unknown etiology is the most typical clinical feature of the Melkersson-Rosenthal syndrome. Its coexistence with of facial nerve palsy or fissured tongue could be characterized as an oligosymptomatic MRS. Many investigators suggest cheilitis gra nulomatosa as a monosymptomatic form of MRS, while patients with facial palsy and fissured tongue, without or of eacial edema, should not be considered having MRS. Histological evidence is not necessary. Corticosteroids are generally accepted as the mainstay treatment.