目的　为提高小儿原发性小肠肿瘤的诊断率 ,以获得早期治疗。方法　对 2 8例原发性小肠肿瘤的病理类型、临床表现、误诊原因、治疗情况作一回顾性分析。结果　小儿小肠原发性肿瘤良性 15例 ,恶性 13例 ,恶性肿瘤以淋巴肉瘤为主 ( 92 .3 % )。术前仅 4例确诊 ( 10 .7% ) ,2 4例术中及术后获得诊断。其中继发性肠套叠而急诊手术 16例 ( 64 .9% )。良性肿瘤除 1例十二指肠血管瘤大出血放弃治疗外 ,14例全部治愈。恶性肿瘤 13例术后虽化疗放疗等治疗 ,经随访目前仅3例存活 1～ 8年。余分别在 2个月至 2年内死亡。结论　小儿原发性小肠肿瘤少见 ,术前诊断困难 ,误诊率高 ,常以肠套叠为首发疾病 ,确诊常在术中及术后获得 ,恶性肿瘤预后差 The purpose is to improve the diagnosis rate of pediatric primary intestinal tumor to obtain early treatment. Methods A retrospective analysis was made on the pathological types, clinical manifestations, causes of misdiagnosis and treatment of 28 cases of primary small intestinal tumors. Results The primary small intestinal intestine was benign in 15 cases and malignant in 13 cases. Malignant tumor was mainly lymphosarcoma (92.3%). Only 4 cases were diagnosed preoperatively (10.7%), 24 cases were diagnosed intraoperatively and postoperatively. Including secondary intussusception and emergency surgery in 16 cases (64.9%). In addition to benign tumor hemorrhage in one case of duodenal hemangioma to give up treatment, 14 cases were cured. Thirteen patients with malignant tumors, although chemotherapy and radiotherapy treatment, after the current only 3 cases survive 1 to 8 years. I die in two months to two years respectively. Conclusions Small intestinal primary tumors in children are rare, difficult to diagnose before operation, high misdiagnosis rate, often with intussusception as the first disease. The diagnosis is often obtained during and after surgery. The prognosis of malignant tumors is poor