Rationale:Intraductal papillary mucinous neoplasms (IPMN) of the accessory pancreatic duct (APD) are very rare and their clinical significance is not known. Here, we describe a case of IPMN originating in a branch of the duct of APD within the uncinate process of the pancreas, which initially presented with acute pancreatitis.Patient concerns:A 70-year-old man visited our medical center presenting with acute pancreatitis around the head and uncinate process of the pancreas.Diagnoses:Computer tomography scans revealed the presence of a 35-mm cystic mass-like lesion within the pancreas uncinate process communicating with a branch of the APD. The patient was diagnosed with APD-IPMN in the pancreas uncinate process accompanied by acute pancreatitis.Interventions:Conservative management of the acute pancreatitis relieved his symptoms, while duodenum-preserving partial pancreatic head resection (DPPHR-P) was performed to treat the APD-IPMN. Intraoperative exploration showed the presence of severe adhesions within the uncinate process of the pancreas and that the tumor’s “peduncle” – a branch of the duct of APD – was saddling just at the front of the main pancreatic ducts. Thus, surgical removal of the tumor required special handling of the region between the main duct (MD) and APD to protect the integrity of the main pancreatic ducts. Finally, a 35*30*15 mm IPMN was successfully removed and the MD was preserved combined with ligation from the root of the APD of the pancreas. The drainage volume of the ventral tube increased by around 20-fold in 24 hours on the fourth day after surgery. The presence of high amylase levels in the drainage discharge (40713.5 U/L) led to the diagnosis of postoperative pancreatic fistula (POPF). The drainage volume remained high for 3 days.Outcomes:The patient was discharged and POPF was successfully managed through endoscopic pancreatic duct stenting.Lessons:APD-IPMN in the pancreas uncinate process has its own characteristics of localized pancreatitis, and MD-preserving DPPHR-P not only protects the exocrine and endocrine functions of the pancreas, but it also protects the physiological and anatomical integrity. The appearance of POPF after DPPHR-P may be managed by endoscopic pancreatic duct stenting.