Small intestinal neoplasms are uncommon cancers. Benign small intestinal tumors (e.g., leiomyoma, lipoma, hamartoma, or desmoid tumor) usually are asymptomatic but may present with complications. Primary malignancies of the small intestine, including adenocarcinoma, leiomyosarcoma, carcinoid, and lymphoma, are often symptomatic and may present with intestinal obstruction, jaundice, bleeding, or pain. Metastatic neoplasms may involve the small intestine via contiguous spread, peritoneal metastases or hematogenous metastases. Because the small intestine is relatively inaccessible to routine endoscopy, diagnosis of small intestinal neoplasms is often delayed for months after onset of symptoms. During last years the increase of small bowel endoscopy and other diagnostic tools allow earlier non-operative diagnosis. Even though radical resection of small bowel cancer plays an important role, the 5 yr overall survival remains low.