目的　探讨原发性小肠恶性肿瘤的临床特点及诊断治疗。方法　对手术后病理证实的原发性小肠恶性肿瘤 2 8例进行分析。结果　病灶位于十二指肠 5例 ,空肠 11例 ,回肠 12例。以平滑肌肉瘤及腺癌居多。常见的临床表现为腹痛、贫血、便血、恶心、呕吐、腹泻、体重下降。术前明确诊断仅 5例 ,占 17.9%。手术中发现转移率 78.6 % ,本组随访率 84.6 %。结论　原发性小肠恶性肿瘤早期症状不典型 ,缺乏特异性阳性体征 ,临床医生应提高对原发性小肠恶性肿瘤的认识 Objective To investigate the clinical features and diagnosis and treatment of primary intestinal malignant tumors. Methods 28 cases of primary malignant tumors of small intestine confirmed by pathology were analyzed. Results The lesions were found in 5 cases of duodenum, 11 cases of jejunum and 12 cases of ileum. To leiomyosarcoma and adenocarcinoma mostly. Common clinical manifestations of abdominal pain, anemia, blood in the stool, nausea, vomiting, diarrhea, weight loss. Clearly diagnosed before surgery in only 5 cases, accounting for 17.9%. Surgery found 78.6% rate of transfer, the follow-up rate of 84.6%. Conclusions Early symptoms of primary small intestinal malignancies are not typical and lack specific positive signs. Clinicians should raise awareness of primary small intestinal malignancies