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Extramedullary plasmacytoma (EMP) of the small intestine is an unusual plasma cell neoplasm in this anatomic region with only 61 cases described so far. Clinical suspicion is infrequent owing to its location and nonspecific manifestations such as abdominal pain, obstructive symptoms or even bleeding. Diagnosis is reached through histopathological examination coupled with immunohistochemistry of the endoscopic biopsy or surgical resection specimens. Nevertheless, the differential diagnosis between EMP, lymphoma and other kinds of tumors can sometimes be troublesome. The managements include surgery, radiotherapy or chemotherapy. Generally, the prognosis of EMP is favorable, but occasionally it may relapse, or progress to a plasma cell myeloma. EMP should therefore be followed-up for a long period after treatment. In this comprehensive review of the current literature, the patients' characteristics, clinical manifestations, diagnosis, differential diagnosis, treatment and outcome were discussed.