Objective: To explore the clinical feature, pathological characteristic, diagnosis and treatment of primary small intestinal choriocarcinoma. Methods: The clinicopathological features of 8 cases of primary small intestinal choriocarcinoma were analyzed and relevant literature were reviewed. Results: Among the 8 cases, the characteristic symptom was melena, and 5 of them were originated from jejunum. An elevated serum β-hCG level was detected in all cases. Microscopically, the tumor consists of cytotrophoblasts and syncytiotrophoblasts, associated with hemorrhage and necrosis. Immunohistochemically, the tumor cells were positive for β-hCG. Conclusion: Although primary small intestinal choriocarcinoma is rare, it should be considered as one of the differential diagnoses. Morphological features, immunohistochemical characteristics and measurement of serum β-hCG level could be used to confirm the diagnosis.