Orofacial granulomatosis(OFG) is a chronic granulomatous inflammatory disorder affecting the mouth and perioral region, typically presenting with swelling of the lips and inflammation of oral mucosa. The etiology remains largely unknown, and the diagnosis and appropriate therapy for OFG has not yet been well defined for all patients. This paper is a review of the epidemiology, etiopathophysiology, diagnosis, clinical manifestations, and treatment in patients with OFG.