ABSTRACT
Agenesis of the dorsal pancreas is a rare congenital anomaly that arises from the failure of the dorsal pancreatic bud of endodermal cells to form the body and tail of the pancreas and can manifest as diabetes. A 24-year-old man, who had been treated with insulin for 7 years, presented with epigastric pain, vomiting, and watery diarrhea. Abdominal computed tomography showed only the head of the pancreas without visualization of the pancreatic body and tail. Left renal agenesis and absence of the left vertebral pedicle in T12 were also observed. The duct of Santorini and the duct in the body and tail were not visible in magnetic resonance cholangiopancreatography. The associated anomalies reported here are very rare globally. We report a case of complete agenesis of the dorsal pancreas with multiple congenital abnormalities and diabetes mellitus.