The Melkersson-Rosenthal syndrome is characterized as a rare neuro-muco-cutaneous clinical syndrome, whose distinctive features include the simultaneous presentation of a triad of clinical signs: recurrent facial paralysis, orofacial edema, and fissured tongue. This combined manifestation is observed in only 8% to 25% of documented cases. The case of a patient diagnosed with Melkersson-Rosenthal syndrome with a two-year history is presented. The presence of the classical triad is noteworthy, and such a comprehensive presentation is infrequently reported in the existing literature. Clinical and histopathological findings associated with the case are discussed.