Complete agenesis of the pancreas is a very rare congenital abnormality presenting with severe intrauterine retardation, persistent hyperglycemia, pancreatic origin malabsorption and other associated malformations. The majority of the few cases presented in the literature were lost during the newborn period, prior to appearance of whole malabsorption signs. Since the definitive diagnosis of complete pancreatic agenesis can be made only by autopsy, delayed diagnosis of this rare abnormality and difficulties in differential diagnosis occur. In this article, we present a case admitted at the age of 27 days with hypoalbuminemia, persistent hyperglycemia and edema who was treated as neonatal diabetes mellitus and subsequently diagnosed by autopsy as pancreatic agenesis in order to emphasize the misdiagnosed congenital pancreatic abnormalities.

Keywords: pankreas agenezisi, hiperglisemi, pancreatic agenesis, hyperglycemia.