BACKGROUND/AIMS In this study, it was reappraised the outcomes of total pancreatectomy (TP), retrospectively analyzing the safety of the procedures and factors associated with long-term survival. METHODOLOGY Thirty-six consecutive patients underwent TP for pancreas disease at Kobe University Hospital. The outcomes of TP were evaluated, analyzing operation-related results (mortality, morbidity, survival and long-term outcomes) and oncological aspects. RESULTS Postoperative morbidity was 39% (14/36) and severe complications were anastomotic leakage (n=3) and liver necrosis (n=1). In benign disease, 5-year survival was 50%, while 5-year survival in malignant disease was 22%. Postoperative glycosylated hemoglobin A1c (HbA1c) level was 7.8 +/- 1.2% at 6 months and 7.8 +/- 1.5% at 12 months after TP, respectively. CONCLUSION TP is safely performed and the treatment option for selectively limited pancreatic cancer and intraductal papillary mucinous neoplasm of the pancreas (IPMN), when the patient condition permits and offers a chance of cure, although careful long-term medical care and follow-up are essential.