AIM: To evaluate the clinical presentation, treatment and survival of patients with primary malignant tumor of small bowel (PMTSB). METHODS: Clinicopathologic data about 141 surgically treated PMTSB patients (91 males and 50 females) at the median age of 53.5 years (range 23-79 years) were retrospectively analyzed. RESULTS: The most common initial clinical features of the patients were intermittent abdominal discomfort or vague abdominal pain (67.4%), abdominal mass (31.2%), bowel obstruction (24.1%), hemotochezia (21.3%), jaundice (16.3%), fever (14.2%), coexistence of bowel perforation and peritonitis (5.7%), coexistence of gastrointestinal bleeding and shock (5.0%), and intraabdominal bleeding (1.4%). Ileum was the most common site of tumor (44.7%), followed by jejunum (30.5%) and duodenum (24.8%). PMTSB had a nonspecific clinical presentation. Segmental bowel resection ( n = 81) was the most common surgical procedure, followed by right hemicolectomy ( n = 15), pancreaticoduodenectomy ( n = 10), and others ( n = 19). Twenty-seven adenocarcinoma patients and 13 malignant lymphoma patients received adjuvant chemotherapy with 5-fluorouracil and cyclophosphamide, adriamycin, vincristine and prednisone, respectively. Information about 120 patients was obtained during the follow-up. The median survival time of PMTSB patients was 20.3 mo. The 1-, 3- and 5-year survival rate was 75.0% (90/120), 40.0% (48/120) and 20.8% (25/120), respectively. Adenocarcinoma was found in 73.7% (42/57), 21.1% (12/57) and 15.8% (9/57) of the patients, respectively. Gastrointestinal stromal tumor was observed in 80.0% (20/25), 72.0% (18/25) and 36.0% (9/25) of the patients, respectively. Carcinoid was detected in 100.0% (15/15), 80.0% (12/15) and 46.7% (7/15) of the patients, respectively. Malignant lymphoma was demonstrated in 69.2% (9/13), 30.8% (4/13) and 0% (0/13) of the patients, respectively. CONCLUSION: En bloc resection is the principal therapy for most PMTSB and chemotherapy is the important treatment modality for malignant lymphoma and other malignant tumors of small bowel which cannot be radically removed.