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We report a case of adenocarcinoma in an intrapancreatic accessory spleen (IPAS). A 78-year-old woman presented with abdominal discomfort, and investigations revealed an elevated serum carbohydrate antigen 19-9 level, to 161.8 U/ml (normal, <37 U/ml). Ultrasonography showed a heterogeneous echogenic tumor with a vascular hilum. Computed tomography showed a heterogeneously enhanced tumor, 8 cm in diameter, adjacent to the pancreatic body, accompanying a feeding artery arising from the splenic artery, and a drainage vein flowing into the splenic vein. We performed a distal pancreaticosplenectomy. The tumor was surrounded by a fibrous capsule and was in contact with the pancreatic body. Histological examinations revealed invasive growth of adenocarcinoma in a structure identical to the spleen. The results of both radiological and histological examinations suggested that the tumor originated from an intrapancreatic accessory spleen. Extensive examinations revealed no other malignancy, based on which we concluded that the adenocarcinoma was primary. Surgical intervention is strongly recommended when a malignancy in an IPAS cannot be ruled out. [ABSTRACT FROM AUTHOR]