Chronic lip swelling may be a clinical sign of a systemic disease, such as Crohn's. We report two cases of children in Greece where the chronic lip swelling was not only the initial, but the sole clinical symptom of Crohn's, and another case with only granulomatous cheilitis up to now. All lip biopsies revealed granulomatous cheilitis. The ileocolonoscopy revealed granulomatous ileitis, and the video capsule endoscopy aphthous lesions in the jejunum in the first two cases. The diagnosis of Crohn's was then established, and treatment was started. In the third case only granulomatous cheilitis was revealed. He was started on minocycline and corticosteroids and remains under frequent monitoring in case he should develop Crohn's. Clinicians should bear in mind that inflammatory bowel disease may present with atypical symptoms. This could thus be the case for any child with persistent lip swelling, and therefore further examinations should be performed to establish the diagnosis.