Context:
Agenesis of the dorsal pancreas is a rare anomaly, mostly associated with other medical conditions. It may be complicated with pancreatic neoplasms.
Case report:
We report the case of a 51-year-old male with selective agenesis of pancreatic isthmus with preservation of main pancreatic duct and branch-duct intraductal papillary mucinous neoplasm (BD-IPMN) with suspicious features of the pancreas.
Conclusion:
This is probably the first report of isolated agenesis of pancreatic isthmus with conservation of main pancreatic duct.