Lymphomatoid granulomatosis is a lymphoproliferative process affecting multiple organ systems usually including the lungs. Significant gastrointestinal involvement, however, has rarely been reported. Pathologic examination reveals a vasocentric polymorphous lymphoid infiltrate. A case of lymphomatoid granulomatosis with gastrointestinal manifestations necessitating aggressive surgical intervention is reported. The clinical presentation, pathologic features, and various aspects of therapy of lymphomatoid granulomatosis involving the gastrointestinal tract are discussed.

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