Total pancreatectomy has been mainly used to treat malignant diseases of the pancreas, di-ffuse ductal involvement, and severe chronic pancreatitis. Patients may develop pancreatic diabetes after the surgery, in whom the glucose levels fluctuate widely and are hard to control, which increases the incidence of recurrent hypoglycemia. Postoperative complications are closely associated with the glucose levels. Thus, tighter glycemic management is essential for increasing the survival of these patients. Few cases have been discribed in China. In this article, we reviewed the blood glucose features, peri- and post-operative complications, and clinical treatment for patients undergoing total pancreatectomy.