Sarcoidosis is a multi-systemic inflammatory disorder of unknown etiology. It is classified as an acquired systemic granulomatous disease. Because of the fact that sarcoidosis affects multiple tissues and organs it is characterized by many potential signs and symptoms, as well as by the presence of noncaseating granulomas in the organs involved. Although orofacial sarcoidosis is relatively rare, it may however, present in the oral and maxillofacial region. The respiratory system is the most commonly affected system, with approximately 90% of patients presenting pulmonary findings during the course of their disease. Cutaneous manifestations occur in around 25% of cases and are more common in chronic cases. Head and neck lesions of sarcoidosis are manifested in 10 to 15% of patients (Samtsov, 1992; Newman et al., 1997; Suresh & Radfar, 2005). In the maxillofacial region the salivary glands may be involved, while sometimes, xerostomia and bilateral parotid swelling may be seen (Piattelli et al., 1998; Batal et al., 1999). Lesions occurring in the soft tissues of the oral cavity and/or in the jaws are rare. Orofacial granulomatosis (OFG) is a granulomatous disease. This clinicopathological entity describes patients with oral lesions characterized by persistent and/or recurrent labial enlargement, ulcers, and a variety of other orofacial features, which on biopsy have lymphedema and noncaseating granulomas. The cause is idiopathic but appears to represent an abnormal immune reaction. This may be a manifestation of Crohn’s disease (CD) since some patients with oral lesions develop typical bowel symptoms of CD in ensuing months to years; tooth associated infections, viruses, food or contact allergies have been implicated in causing OFG. Sarcoidosis has also been implicated in causing OFG. Clinical features of OFG are highly variable and sometimes so insidious that signs and symptoms are frequently not severe enough to cause alarm. The lips are most commonly involved and demonstrate a nontender, persistent swelling. Because of the relatively nonspecific clinical findings associated with granulomatous diseases, a microscopic diagnosis of granulomatous inflammation per se often presents a diagnostic dilemma (Shams et al., 2007).