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Lymphomatous involvement of the appendix is rare and most of them are found accidentally from appendectomies to treat appendicitis. Here we report three cases of primary lymphoma of appendix of 10 year-old boy, 23-year-old man and 24-year-old woman that presented with abdominal pain to our hospital and the tumors were discovered during routine appendectomy for suspected "appendicitis". Our cases underwent chemotherapy regimen and they were asymptomatic respectively at 14, 17, and 18 months following-up. In the literature review of the tumors of the appendix, most of the begin tumors of appendix are treated with surgery alone. Lymphoma requires CHOP-like chemotherapy and carcinoid tumor treated according to the size of the tumor <2 with appendectomy alone and >2 with right hemicolectomy. The preferred treatment for adenocarcinoma of appendix is right hemicolectomy and the role of chemotherapy is unclear but when lymph node involvement is present, chemotherapy seems to be accepted. [ABSTRACT FROM AUTHOR]