Malignant tumors of the small intestine are rare and difficult to diagnose. The difficulty in early diagnosis often brings poor prognosis to the patients. In western countries, lymphoma accounts for only a small part of small intestinal malignancies, but in Taiwan, it is the most common type of malignant small intestinal tumors. We report a patient of small intestinal lymphoma, presenting with chronic diarrhea and body weight loss. The clinical symptoms, tumor location, age of onset, result of serum immunoelectrophoresis, and development back-ground of this patient are all compatible with the so called “Mediterranean lymphoma”. The diagnosis of this case was made by high index of suspicion and was confirmed by endoscopic biopsy. The importance of endoscopy in the diagnosis of small intestinal tumors is emphasized.