An 18-year-old girl was referred to the Department of Oral Medicine with upper lip swelling. She was in good general health and laboratory tests were within the normal range. Histopathologic diagnosis did not reveal the presence of granulomas. This is consistent with the finding that 30% of patients with orofacial granulomatosis do not have granulomas on their biopsies. The patient was treated with intralesional steroids once a week for three weeks. The lesion subsided, but not completely, and recurred partially after ketchup intake. This case report highlights the fact that in every patient with non-odontogenic facial or oral swelling, systemic diseases such as sarcoidosis, Crohn's disease, tuberculosis, etc. must be excluded. Furthermore, it is not unusual that in patients with orofacial granulomatosis, noncaseating granulomas are absent in the histopathologic finding.