Background: Despite improvements in therapy regimens over the past decades, overall survival rates for pancreatic and periampullary cancer are poor. Specific cancer registries are set up in various nations to regional differences and to enable larger prospective trials. The aim of this study was to describe the Swedish register, including possibilities to improve diagnostic work-ups, treatment, and follow-up by means of the register. Methods: Since 2010, all patients with pancreatic and periampullary cancer (including also patients who have undergone pancreatic surgery due to premalignant or benign lesions) have been registered in the Swedish National Periampullary and Pancreatic Cancer registry. Results: In total 9887 patients are listed in the registry; 8207 of those have malignant periampullary cancer. Approximately one-third (3282 patients) have had resections performed, including benign/premalignant resections. 30-day and 90-day mortality after pancreatoduodenectomy is 1.5% and 3.5%, respectively. The overall 3-year survival for resected pancreatic ductal adenocarcinoma is 35%. Regional variations decreased over the studied period, but still exist. Conclusion: Results from the Swedish National Registry are satisfactory and comparable to international standards. Trends over time show increasing resection rates and some improved results. Better collaboration and openness within pancreatic surgeons is an important side effect.