The jejunum and ileum neoplasms, in addition to rare diseases, have some difficulty of suspicion due to an arsenal of nonspecific signs and symptoms, responsible for diagnostic delay main. Adenocarcinomas, carcinoid tumors, lymphomas and sarcomas are tumors of evil nature, while adenomas, leiomyomas, fibromas and lipomas make up the benign. The general principle of treatment of primary small intestine malignant blastomas is resection with free surgical margins, associated or not to locoregional lymphadenectomy, depending on the histological type and surgical staging of neoplasia.