Granulomatous cheilitis in the paediatric population may be an initial manifestation of Crohn’s disease. The article reports the case of a 13-year-old boy who presented with epigastralgia and fever for ten days. Nine months before he had been diagnosed granulomatous cheilitis that had been treated with intramuscular corticosteroids with only partial healing. An increase in ESR, C-reactive protein test (CRP) and faecal calprotectin were observed. P-ANCA and ASCA were positive. Abdominal ultrasonography revealed the parietal thickening of the ileal handle with mesenteric lymph nodes of increased size. Esophagusgastroduodenoscopy and ileocolonscopy showed macroscopic and histological findings that were compatible with Crohn’s disease. 8 weeks of exclusive oral polymeric diet treatment, in association with azathioprine after 2 weeks from the beginning of the diet, led to clinical remission and faecal calprotectin, ESR and CRP normalization.