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The stomach is the most common site of gastrointestinal tract lymphoma, while synchronous mucosa-associated lymphoid tissue(MALT) lymphomas of the stomach and duodenum are very rare. A literature review found no reported case of synchronous gastroduodenalMALT lymphomas with involvement of the bone marrow and spleen. Here, we describe the case of a 62-year-old malewho was diagnosed with synchronous MALT lymphomas of the stomach and duodenum based on upper gastrointestinal endoscopyand pathology. Other staging evaluations, including colonoscopy, abdominopelvic and chest computed tomography (CT), 18F fludeoxyglucose-positron emission tomography (FDG-PET), and a bone marrow examination, showed involvement of the bone marrowand spleen. We diagnosed stage EIV MALT lymphoma and began systemic chemotherapy. We report the first case of MALTlymphomas arising synchronously in both the stomach and duodenum with bone marrow involvement and review the literature.