AIMS: Jejunoileitis might be a severe form of Crohn's disease (CD). The aim of the study was to evaluate clinical characteristics, therapeutics modalities and long-term outcome in CD patients with jejunoileitis (CDJI). METHODS: All patients with CDJI followed in the department of Gastroenterology from 1963 to 1999 were included and compared to matched (on Year of CD diagnosis) CD controls without jejunoileitis. Data were obtained from retrospective review of medical charts. RESULTS: Eighteen patients with CDJI were compared to 36 matched CD controls. Median follow-up was 7.65 Years in both groups. At time of CDJI diagnosis the following signs were significantly more frequent in patients with jejunoileal CD than in controls: malnutrition (39% vs 3%), pain suggesting obstruction (33% vs 8%), vomiting (28% vs 5%). Patients with CDJI were more frequently male: M/F ratio=2.0/1.1 (P=0.33). Upper digestive involvement (esophagus, stomach and duodenum) (67% vs 36%, P=0.04) and small intestine strictures (61% vs 19%, P=0.06) were more frequent in CDJI. Initial management was more "aggressive" in CDJI than in controls: steroids in 62% vs 30%, azathioprine in 39% vs 3%, total parenteral nutrition in 28% vs 8% and surgery in 33% vs 17%. During follow-up, the need for azathioprine therapy and surgery were more frequent in CDJI than in controls (extensive small bowel resection in two patients). In 10 of 18 patients, jejunoileitis involvement was diagnosed with a median delay of 3.6 Years (range: 0.5-14.5) after CD diagnosis and at time of CD diagnosis in the 8 others; outcome after CDJI diagnosis was similar in these 2 groups. CONCLUSION: The main revealing signs of jejunoileitis in CD patients are obstruction and malnutrition. Patients with CDJI require more often azathioprine and surgery than CD patients without jejunoileitis. Jejunoileitis is a severe form of CD more frequently complicated by extensive small bowel resection.