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The variable clinical presentation of heterotopic pancreatic (HP) tissue and its malignant transformation makes the diagnosis very challenging. These lesions are very rare, usually not suspected upon initial presentation and for the most part, are diagnosed incidentally on review of pathology following surgical resection. In this study, we are reporting an adenocarcinoma arising from jejunal heterotopic pancreatic tissue in a 59-year-old female and a brief review of previously reported cases. [ABSTRACT FROM AUTHOR]