An elevation of serum AFP was pointed out in a 64-year-old man during a follow-up for liver cirrhosis and diabetes mellitus. Abdominal CT showed a mass in the head of the pancreas, and he was referred to our department. Endoscopic ultrasound-guided fine needle aspiration biopsy revealed class V disease (adenocarcinoma) , and we made the diagnosis of pancreatic cancer. We performed total-pancreatectomy, because the distal margin of the pancreatic duct was positive. After the operation, the serum AFP level became normal. We finally diagnosed this tumor as AFP producing intraductal tubulopapillary neoplasm (ITPN). The patient showed no recurrence at 24 months after the operation.