Dorsal pancreatic agenesis is an extremely rare entity characterised by absence of body and tail of pancreas, while there are so many other developmental anomalies of the pancreas that have been reported. Here we report a 25-year-old young man who presented with pain in the abdomen, recurrent loose stools and hyperglycaemia. On radiological imaging study, there was complete agenesis of the dorsal pancreas except for thin stripe of tissue at the level of the uncinate process. Both exocrinedysfunction and endocrine dysfunction were present in this patient. Patient was supplemented with pancreatic enzyme preparation and insulin.
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