Total pancreatectomy with segmental duodenectomy including major and minor papilla and preservation of the gastroduodenal artery is performed for low-grade malignancy tumor of the whole pancreas. Reconstruction of the alimentary tract was done by end-to-end duodenoduodenostomy and end-to-side choledochoduodenostomy. This is an easy, simple, safe and function-preserving operative procedure for benign or low-grade malignancy tumors of the whole pancreas.