Orofacial granulomatosis is an uncommon clinicopathological entity describing patients who have oral lesions characterized by persistent and/or recurrent labial enlargement, oral ulcers and a variety of other orofacial features, who on lesional biopsy have lymphoedema and non-caseating granulomas. The aetiology of oral lesions with non-caseating granulomas includes oral Crohn's disease (some patients with oral lesions will develop typical bowel symptoms of Crohn's disease in ensuing months to years), tooth-associated infections, sarcoidosis and food or contact allergies. Treatment of orofacial granulomatosis is not reliably effective and may not be always necessary, although most patients do require some medical intervention.