Partial or complete agenesis of the dorsal pancreas is a rare congenital anomaly that results from the embryological failure of the dorsal pancreatic bud to form the body and tail of the pancreas. To date, four cases have been reported in Korea. We report an additional case; a 25-year-old woman presented with diabetes mellitus and abdominal pain. Abdominal computed tomography (CT) revealed a normal-appearing pancreatic head, but the body and tail were not visualized. Endoscopic cholangiopancreatogram (ERCP) revealed a short pancreatic duct in the uncinate process and the head and the duct of Santorini draining into the minor papilla. Abdominal magnetic resonance imaging (MRI) findings were similar to the CT and ERCP results. The patient was diagnosed with partial agenesis of the dorsal pancreas by CT, ERCP and MRI.