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Intraductal papillary mucinous carcinoma (IPMC) arising from the heterotopic pancreas is rare. A case of IPMC metastasis from the jejunal heterotopic pancreas was described. The heterotopic pancreas could be the source of the submucosal tumor-like lesion found in the small intestine with an elevated carbohydrate antigen (CA) 19-9 level. A 60-year-old woman was admitted to the hospital with pulmonary thromboembolism and anemia. The level of CA19-9, a tumor marker, was found to be 211.8U/ml. A tumor in the jejunum was discovered using contrast-enhanced computed tomography. There were also a number of hepatic tumors found. A submucosal tumor-like lesion in the jejunum was discovered during an enteroscopy, and a biopsy revealed it to be an adenocarcinoma. Partial resection of the jejunum was performed to control hemorrhage. Histopathology revealed an invasive IPMC arising from a heterotopic pancreas (Heinrich type II) and chemotherapy with gemcitabine and nab-paclitaxel was initiated. There have only been three cases of invasive IPMC from a heterotopic pancreas reported, and this is the first one to include chemotherapeutic treatment of distant metastasis.