Introduction Disconnected pancreatic duct syndrome (DPDS) is a pathological condition that causes various symptoms due to the continuous secretion of pancreatic enzymes from the pancreas upstream, which has been separated due to disconnection of the pancreatic duct (DPD) for various reasons.Acute necrotizing pancreatitis includes a certain probability of DPDS appearance, which makes it necessary to provide various treatments for DPDS. Furthermore, DPDS can impact long-term results, such as recurrence and impaired pancreatic function. Although the development of various modalities has contributed to diagnosis and treatment, especially less invasive endoscopic therapy, DPDS is often overlooked, and the diagnosis can be delayed due to the lack of consensus on its definition and classification. This review summarizes the current knowledge and challenges of DPDS and discusses the optimal strategy for its diagnosis and treatment, as well as future perspectives.