The descriptive term ‗Orofacial Granulomatosis‘ has been used for any granulomatous process of unknown etiology involving the oral cavity, which on lesional biopsy shows lymphodema and non- caseating granulomas. The diagnosis is based on exclusion of possible systemic diseases and conditions that are capable of producing similar histopathologic and clinical features. Orofacial granulomatosis can be a distinct clinical disorder or can be an initial presentation of an underlying systemic disease such as Crohn‘s disease or spectrum of related disorders that may go undetected for a long period of time.