Background: Total pancreatectomy (TP) is not more beneficial than less aggressive resection techniques for the treatment of pancreatic neoplasms and is associated with high morbidity and mortality. However, with advances in surgical techniques and glycemic monitoring, and the development of synthetic insulin and pancreatic enzymes for postoperative treatment, TP has been increasingly indicated. This is a review of the recent literature reporting the clinical outcomes after TP. Methods: We reviewed the publications reporting the use of TP starting 2007. The clinicophysiological and survival data were analyzed. Results: Few studies evaluated the differences in clinical outcomes between TP and pancreaticoduodenectomy (PD) with inconsistent results. It was reported that while the perioperative morbidity did not decrease, the mortality decreased compared to previous literature. All patients who underwent TP required insulin and high dose of pancreatic enzyme supplements. The 5-year survival rates after TP and PD for pancreatic cancer were similar. Conclusion: The perioperative mortality decreased in patients who underwent TP with advances in the operative procedures and perioperative care. The long-term survival rates were similar for TP and PD. Therefore, treating pancreatic neoplasms using TP is feasible. Patients undergoing TP should receive adequate treatment with synthetic insulin and pancreatic enzyme supplements.