We report a rare case of common bile duct mucosa-associated lymphoid tissue (MALT) lymphoma treated with pancreatico-duodenectomy with a partial gastrectomy. MALT lymphoma involving the biliary tree is extremely rare. Diagnosis is difficult and treatment options are controversial. Even though Helicobacter pylori treatment is effective in the early stages of the disease, surgery is still helpful especially when obstruction, perforation, or bleeding is present.
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