Objective: To study the clinical features, diagnosis and management of primary jejuno-ileal carcinoma and to analyze its main prognostic factors. Methods: The clinical, pathological and follow-up data of 14 patients with primary jejuno-ileal carcinoma, admitted to our hospital from January 2000 to January 2008, were retrospectively analyzed. Results: Carcinoma of the jejunum and ileum occurred in both men and women, and the ratio of men to women in our data was 1∶6. The main clinical symptoms of the patients comprised abdominal pain, abdominal distension, hematochezia, anemia, abdominal mass and intestinal obstruction. Of the patients, 9 cases had jejunal carcinoma and 5 cases had ileal carcinoma. Thirteen cases underwent tumor resection, of which 5 cases received 2- to 6-cycle of postoperative chemotherapy. One case, who had liver metastases and ascites, only underwent biopsy and subsequent supportive therapy. Of the whole group, the preoperative diagnotic rate was less than 50% and the 3-year survival rate was 50%. The better chance of survival of the patients after surgery was associated with better differentiated tumor type, less involvement of lymph nodes, and lesions distant from the ligament of Treitz, and vice versa. Conclusions: The preoperative dagnosis of carcinoma of the jejunam and ileum is difficult and requires the B-ultrasounic, CT and endoscopic examinations. Surgical resection is still the main treatment method of this disease and the patients may have a better chance of survival after surgery.