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A case of pancreatic somatostatinoma with duodenal metastasis is reported. The presenting feature was severe right hypochondrial pain with cholecystolithiasis and mild diabetes mellitus. Duodenogastroscopy revealed a small polypoid lesion at the duodenal peri‐ampulla of the major papilla. A biopsy specimen demonstrated a neuroendocrine tumor. Selective gastroduodenal arteriography disclosed a spherical hypervascular lesion in the pancreaticoduodenal arcade. Pancreatoduodenectomy was performed and pathological examination demonstrated pancreatic islet cell tumor and metastasis to the duodenal peri‐ampulla. Immunocytochemical examinations demonstrated intra‐cytoplasmic somatostatin and calcitonin in both tumors. The patient's basal plasma somatostatin level was 4 times higher than the normal range, however her plasma calcitonin level was within the normal range. After the operation, plasma somatostatin levels were within the normal range and her clinical course was good. In this case endoscopy was very useful in detecting the early stage duodenal metastasis of pancreatic somatostatinoma.

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