Orofacial granulomatosis (OFG) is a rare clinic pathological entity of unknown etiology, which appears in patients with oral lesions in the form of persistent or recurrent lip enlargement, oral ulcerations and another symptoms in the orofacial area. Histologically it is characterized by noncaseating granulomas in lamnia propria. The diagnosis of OFG is based on the exclusion of systemic diseases, such as Chron's disease, sarkoidosis, tuberculosis etc. The treatment is a challenge considering the fact that no matter what method of treatment is selected, the disease is prone to recurrences. This case report describes an eighteen year old girl that was referred to the Department of oral medicine with upper lip swelling. She was in good general health and laboratory tests were within normal range. A histopathological diagnosis did not reveal the presence of granulomas. This is consistent with the findings that 30% or more patients with OFG do not have granulomas on their biopsies. The patient was treated with intralesional steroids once a week during the period of three weeks. The lesion subsided, but not completely. Lip swelling recurred partially after intake of ketchup. This case report highlights the fact that in every patient with nonodontogenic facial or oral swelling, systemic diseases, such as sarcoidosis, Chron's disease, tuberculosis etc. must be excluded.