Hepatic adenoma is a well-defined, benign, solitary tumor of the liver. In individuals with glycogen storage disease I, adenoma tends to occur at a relatively younger age and can be multiple (adenomatosis). Imaging plays a pivotal role in diagnosing hepatic adenoma and in differentiating adenoma from other focal hepatic lesions. Especially in patients with von Gierke disease, in addition to the associated hepatomegaly caused by steatohepatitis and the diffusely reduced attenuation of the liver parenchyma seen on CT, there may be more than one hepatic adenoma in up to 40% of patients. Malignant degeneration of hepatic adenoma into hepatocellular carcinoma can occur and hence imaging is important for prompt diagnosis of adenoma and its complications. In this case report, we present a case of liver adenoma diagnosed by CT scan in a patient with von Gierke disease.