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Orofacial granulomatosis is an uncommon disorder, but has been increasingly recognized in the past decade. It causes significant morbidity in the patient including oral ulcerations, enlargement of soft tissues which are often persistent and painful. Orofacial granulomatosis is a term generally used to describe lip swelling secondary to an underlying granulomatous inflammatory process.Granulomas are seen in a wide variety of diseases, both infectious and non-infectious. Infections that are characterized by granulomas include tuberculosis, leprosy, histoplasmosis, cryptococcosis,coccidioidomycosis, blastomycosis and cat scratch disease. Granulomatous cheilitis is the histopathological description of such inflammation occurring in the lips and surrounding tissues. Oral Crohn's disease also belongs to the entity of orofacial granulomatosis. Granulomatous diseases cause a great range of symptoms not only in the head and neck but alsothroughout the body. We as oral physicians should beable to diagnose all the granulomatous disease properly for a good treatment and prognosis.