Background: Improved safety of pancreatic surgery has led to consideration of more aggressive approaches, such as resection for primary pancreatic ductal adenocarcinoma (PDAC) with metastatic disease (M1). Methods: A total of 29 patients who underwent pancreatic resection with resection of associated metastatic disease (interaortocaval lymph node dissection, liver resection, and/or multiorgan resections) were retrospectively identified from a database of 316 R0/R1 pancreatic resections for PDAC. An explorative data analysis of perioperative and clinicopathological parameters, and overall survival was performed by Kaplan-Meier estimation, log rank test, and Fisher's exact test. Results: The overall in-hospital mortality and morbidity of R0/R1 pancreatic resections for M1 disease (n = 29) was 0% and 24.1%, compared with 4.2% and 35.2% of R0/R1 pancreatic resections for M0 disease (n = 287). The median overall survival time was 13.8 months (95% confidence interval [CI], 11.4-20.5), and the estimated 1-year overall survival rate was 58.9% (95% CI, 34.8-76.7) for patients with M1 disease. The median survival in those with metastatic interaortocaval lymph nodes was 27 months (95% CI, 9.6-27.0), whereas it was 11.4 months (95% CI, 7.8-16.5) and 12.9 months (95% CI, 7.2-20.5) for those with liver and peritoneal metastases, respectively. Conclusions: Pancreatic resections with M1 disease can be performed with acceptable safety in highly selected patients. The survival after interaortocaval lymph node resection is comparable to that of other lymph nodes that do not constitute M1 disease. Resection of liver and peritoneal metastases, although safe in this series, cannot be generally recommended until further controlled trials can be conducted.