Glycogen hepatopathy is the pathological overloading of hepatocytes with glycogen that is associated with poorly controlled diabetes mellitus. Clinically, it presents with abdominal discomfort, tender hepatomegaly and elevated transaminases. In this report, we describe our experience with two cases of type I diabetes mellitus and glycogen hepatopathy. The patients underwent isolated pancreas transplantation, following which, we have been able to demonstrate complete histological resolution of glycogen hepatopathy associated with control of glucose metabolism.