Since patients with IPMN of the pancreas are at risk of developing recurrent IPMN and pancreatic ductal adenocarcinoma in the remnant pancreas and extrapancreatic malignancies, early recognition, treatment, and systemic surveillance are of great importance. No conclusions can be drawn from the available evidence with respect to the efficacy of surveillance and follow-up treatment programs. A better understanding of the natural course of IPMN and the biology of pancreatic cancer is mandatory to enable further diagnostic and treatment improvements.