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Context Agenesis of the dorsal pancreas is a rare developmental anomaly. This anomaly may be complicated by recurrent acute and chronic pancreatitis.Case report We report the case of a 28-year-old female with agenesis of the dorsal pancreas and chronic calcific pancreatitis. The diagnosis of agenesis of the dorsal pancreas is discussed and the genetic changes leading to it are reviewed. The possibility of the patient having tropical pancreatitis is mentioned.Conclusions This is probably the first report of chronic calcific pancreatitis complicating agenesis of the dorsal pancreas.