Total pancreatectomy was performed for carcinoma of the head of the pancreas associated with multiple anomalies in the peripancreatic region and of the pancreas. The anomalies were preduodenal portal vein, annular pancreas with agenesis of the dorsal pancreas, left-sided gallbladder, polysplenia and high mobile right colon. The surgical implications of pancreatectomy for such anatomical abnormalities, especially preduodenal portal vein, and the usefulness of ultrasonography for the preoperative evaluation are given attention.