Abstract: Objective: To explore the pathological type, clinical characteristics and diagnosis of primary small intestinal tumor (PSIT). Methods: Retrospective analysis of the clinicopathological data for 53 cases with primary small intestinal tumor was conducted. Results: Among the cases, there were 11 with benign small intestinal tumor and most of the benign tumors were leiomyomas (27.3%). There were 42 malignant small intestinal tumor cases. Interstitial sarcoma was the most frequently seen malignant PSIT (40.5%) and adenocarcinoma was the second most frequent (35.7%). Gastroduodenoscopy was performed in 18 cases, of which the results of 9 were positive. Computerized tomography (CT) was conducted for 32 cases and 25 positive results were found. Conclusions: To enhance the diagnostic rate of PSIT, parallel evaluation using gastroduodenoscopy, CT, selective arteriography and endoscope retrograde cholangiopancreatography (ERCP) is needed.