[Type IA glycogenosis with acute pancreatitis]

Type IA glycogenosis, or von Gierke disease, is the most common among the glycogenoses with enlarged liver. Acute pancreatitis is a rare manifestation of type IA glycogenosis and has been attributed to elevated serum fat levels. We report a case of type IA glycogenosis with acute pancreatitis. The radiologists should be familiar with the computed tomography findings in this rare complications of type IA glycogenosis.