Abstract: Objective：To report a case of cryptogenic multifocal ulcerating stenosing enteritis (CMUSE), and to improve the cognition of this rare disease. Methods：Clinical characteristics, laboratory examination, imaging examination, endoscopic findings, pathological features and treatment of the patient with CMUSE from Children's Hospital of Fudan University were collected. The related literature was searched from Wanfang Data Service Platform, Chinese Biomedical Database, PubMed and EMBASE (up to June 2018) by using search terms ("cryptogenic multifocal ulcerating stenosing enteritis" OR CMUSE OR "cryptogenic, multifocal, ulcerous, and stenosing enteritis"). Results：We reported a male child who presented with repeated anaemia and melaena for more than 6 years. Capsule endoscopy of multifocal ulcerating stenosing and pathological features were compatible with CMUSE. Systemic corticosteroid was initiated at 2 mg·kg-1·d-1. The side effects of systemic corticosteroid about visual ambiguity were observed after one month. Then, the steroid was weaned, and azathioprine was initiated at 1 mg·kg-1·d-1 as a steroid-sparing agent. After following for 8 months, the patient was in the remission without anaemia, melaena and abdominal pain. According to literature, 68.6% of cases had an onset in youth and middle-age adults, and the main clinical manifestations were abdominal pain (67.9%), anaemia (32.1%) and gastrointestinal bleeding (18.5%). Capsule endoscopy had a risk retention rate of 37.9%, and the recurrence rate was high with the reoperation rate of 29.7%. Conclusion：The diagnosis of CMUSE should be considered in patients with unexplained recurrent melaena, anemia and small intestinal ulcers and stenosis. Endoscopy plays a vital role in the diagnosis. Glucocorticoid is effective, but easy to relapse.