Melkersson-Rosenthal syndrome (MRS) has been described as a rare disease. Localized swelling (usually of the lips) with noncaseating epithelioid granuloma is necessary for diagnosis. We report 11 patients with biopsy-proven MRS. Mean age was 28 years (range 12-47 years). Mean duration of lesions was 4.4 years. Lip biopsy confirmed typical sarcoid-like granuloma. Five additional patients with typical symptoms are awaiting or refused lip biopsy. Clinically all patients presented with localized swelling of the lips. Treatment with clofazimine (100 mg 4 times weekly for 3-11 months) was associated with complete remission in 5/10 patients and clinical improvement in 3/10 patients with moderate constant swelling. Two patients (one severe and one moderate swelling) failed to respond to treatment. Rebiopsy in four patients confirmed improvement with disappearance or decreased granulomatous inflammation in 3/4. Complete spontaneous remission was also seen in three untreated patients. We conclude MRS is not uncommon, clofazimine may be effective treatment in moderate lesions and spontaneous remissions can occur.