Aim. To determine the indications and to evaluate early and long-term outcomes of total pancreatectomy for pancreatic cancer. Material and methods. Treatment of 29 patients who underwent one- and two-stage pancreatectomy for different malignancies was analyzed. Results. Median of surgery duration and intraoperative blood loss was 280 min and 2200 ml respectively. Postoperative complications were observed in 9 (31%) patients. There were 2 (6.9%) deaths. 1- and 3-year overall actual survival was 61% and 16% respectively in case of ductal adenocarcinoma. Median was 18 months. Patients after surgery for primary multiple lesion (cancer of pancreatic body-tail and major duodenal papilla), pancreatic metastases of renal cancer, mucinous cystadenoma and neuroendocrine cancer are still alive. Follow-up periods are 4, 49, 49 and 65 months respectively. Conclusion. Total pancreatectomy is difficult intervention followed by severe metabolic disorders. However it can improve long-term survival with acceptable incidence of postoperative complications and quality of life if clear indications for surgery are present.