A triad of facial palsy, facial edema, and furrowed tongue characterizes 
Melkersson-Rosenthal syndrome, a rare, noncaseating granulomatous disease of 
unknown cause. Although most reported cases of Melkersson-Rosenthal syndrome 
involve swelling of the perioral area, the authors present a case of 
Melkersson-Rosenthal syndrome involving the periocular area. Because of its 
rarity, the syndrome is usually ignored and misdiagnosed; however, the syndrome 
should not only be considered in the classic perioral presentation but also in 
the rare periocular form, which may be confused with orbital tumors and orbital 
pseudotumors. Biopsies should be performed routinely in all patients who present 
with eyelid edema of unknown etiology. The physician and surgeon who see 
patients with head and neck pathology should be familiar with 
Melkersson-Rosenthal syndrome, and with the possibility of its presentation in 
the orbit and periocular region.