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Although the presence of pancreatic tissue outside of the usual anatomical location of the pancreas is not an uncommon incidental finding, the risk of malignancy is extremely low. We report a case of ductal adenocarcinoma arising within a focus of heterotopic pancreas, occurring in the jejunum of an 81 year old woman. The patient presented with a history of progressive gastrointestinal occlusive symptoms. Computerized tomography showed a mass in the jejunum, which was surgically removed. Histopathology revealed an invasive pancreatic ductal adenocarcinoma from pancreatic tissue with no connection with the original gland (Heinrich type II). At the 12-month follow-up, there were no signs of recurrence. Despite the low risk of malignancy in heterotopic pancreas, adenocarcinoma is the most frequent histological type, and the prognosis is still not clear. Lesions incidentally detected during surgery and in symptomatic patients need to be removed by conservative procedures in order to exclude malignant disease.