Melkersson-Rosenthal syndrome (MRS) is defined by the triad of labial edema, peripheral facial paralysis, and plicated tongue. It is a complex entity with clinical polymorphism and unknown etiopathogenesis. A better understanding of the pathophysiological mechanisms would improve the management of these patients, who often present with significant aesthetic damage. We report a rare case of this syndrome with a plicated tongue and sequellar lagophthalmos.