A case of nonfunctioning islet cell carcinoma was reported. A 40-year-old woman was admitted with epigastralgia. Abdominal angiography and other diagnostic modalities suggested pancreatic malignancy. After distal pancreatectomy, histopathological study revealed her pancreatic tumor to be a nonfunctioning islet cell carcinoma. Fourteen years later, postoperative computed tomographic examination (CT) detected the recurrence of para-aortic lymph node metastases. Five years later, distal gastrectomy was performed to control bleeding from a gastric ulcer. Twenty-one years after the original operation, she died because of underlying metastatic carcinoma. In this case, slow growth and a low grade malignancy were characteristic. Operative removal of the tumor would be the treatment of choice even if metastatic lesions existed.