Glycogenic hepatopathy (GH) is a rare and under-recognized complication in patients with long-standing poorly controlled diabetes mellitus (DM). It was first described by Mauriac, in 1930, in diabetic children.1 The key feature is glycogen accumulation in the liver causing hepatomegaly and raised serum transaminases with wide fluctuations in both glucose and insulin levels. It is important to distinguish this entity from other causes of hepatomegaly and elevated liver enzymes in diabetics, such as nonalcoholic fatty liver disease. Persistent and relatively mild disturbance in liver function favours nonalcoholic fatty liver disease whereas transaminase flares are more compatible with GH. The hallmark of this condition is its reversibility with improved glycemic control, unlike other conditions such as hepatic steatosis. Furthermore, glycogen overload is not known to progress to fibrosis, thus it is distinguishes from fatty liver disease. However, a final distinction can be made with a liver biopsy.