BACKGROUND/AIMS:The difficulty in establishing early definitive diagnosis and treatment of primary small bowel malignancy (PSBM) is a challenge for clinicians. This study aimed to analyze the symptomatology, diagnosis and outcomes of PSBM. METHODOLOGY:A retrospective chart review study was conducted on 49 patients who underwent surgical treatment for PSBM at a tertiary hospital between May 2000 and January 2010. RESULTS:The patients with PSBM had non-specific clinical symptoms. Major diagnostic tools in the current study were computed tomography (CT) (n=40), gastrointestinal series radiography (n=10), angiography (n=5), double-balloon enteroscopy (n=3), gastroduodenoscopy (n=2), magnetic resonance imaging (n=1) and exploratory laparotomy (n=8). Gastrointestinal stromal tumor (GIST) was the most common PSBM (n=21), followed by lymphoma (n=14), adenocarcinoma (n=8), leiomyosarcoma (n=3) and sarcoma (n=3). The overall 5-year cumulative survival rate of the patients with PSBM was 57.1% with a mean survival time of 31.8±7.8 months. The 5-year cumulative survival rates for the different PSBMs were 79.1%, 46.9% and 33.2% for GIST, adenocarcinomas and lymphomas, respectively (p=0.087). CONCLUSIONS:CT is very useful in detecting PSBM. Poor prognosis in PSBM patients detected by CT or angiography could be the result of delayed diagnosis prior to the procedure.