Melkersson-Rosenthal syndrome (MRS) is a rare neuromucocutaneous granulomatous disorder classically defined by the triad: fissured tongue (lingua plicata), recurrent orofacial edema, and relapsing facial paralysis. Association with other dysimmune disorders was reported suggesting an immunological origin to this syndrome. The association with autoimmune thyroiditis remains exceptional and unusual. We report the original case of an MRS associated with Hashimoto autoimmune thyroiditis in a 39-year-old Tunisian man with favorable outcome under thyroxine and systemic glucosteroids. A dosage of thyroid hormones and a screening for anti-thyroid antibodies would be useful in patients with an MRS.