INTRODUCTION: Small bowel presents 75% of the gut length and 90% of the gut surface. However, primary malignant tumors of the small bowel represent only 1-3% of all malignant gastrointestinal tumors. The aim of the present paper is to offer a current review of primary malignant small bowel tumors - their epidemiology, localization, symptoms, diagnostic and treatment options. METHODS: The authors have performed a comprehensive review of databases Medline, Scopus and Google Scholar focusing on studies regarding small bowel cancer. RESULTS: The most frequent small bowel tumors are adenocarcinoma (30-40%), neuroendocrine tumors (35-44%), lymphomas (10-20%) and gastrointestinal stromal tumors (12-18%). Symptomatology is non-specific and varies widely, which is why small bowel cancer is usually diagnosed in a locally advanced stage of the disease. Diagnosis is determined through standard methods (gastroscopy, colonoscopy, CT) and complementary special diagnostic modalities (capsule enteroscopy, enteroscopy, octreotide scan, etc.). Diagnostic process with a negative outcome frequently leads to diagnostic laparoscopy/laparotomy.The treatment of small bowel cancer in patients operated in acute settings is done according to acute abdomen management guidelines. Elective surgery of small bowel cancer differs with respect to the tumor type. Adenocarcinomas and neuroendocrine tumors should be treated with surgical R0 resection with radical lymphadenectomy (and multivisceral resection if necessary). Patients with GIST should undergo en bloc resection with 2-3cm safety resection margins (lymphadenectomy is not necessary). Palliative resection of neuroendocrine tumors can be associated with a significant clinical effect. On the other hand, palliative resection of adenocarcinomas of GIST is not advocated. CONCLUSION: Small bowel cancer is an infrequent condition. Symptoms are non-specific; patients are often diagnosed in an advanced stage of the disease. Achieving R0 surgical resection is usually difficult due to locally advanced stage of the disease. Besides the tumor type, patients prognosis is influenced by very late diagnosis of the tumor. KEY WORDS: primary tumor - small intestine - diagnostics - treatment options - surgical resection.