Glycogen storage disease type I is a rare autosomal recessive inherited disorder with an annual incidence of approximately 1:100,000 [1]. Due to a deficiency of glucose-6-phosphatase [2], glycogen stored in the liver cannot be metabolized. This leads to poor tolerance to fasting and increased risk of hypoglycemia and lactate acidosis. The accumulation of glycogen [3] in liver tissue leads to hepatomegaly, and later in life to an increased risk of hepatocellular adenoma and/or carcinoma. The clinical presentation is accompanied by growth retardation. Renal affection, hyperlipidemia [4], and platelet dysfunctions [5] are common. Perioperative management has to focus on metabolic homeostasis by adequate glucose supply and prevention of lactate acidosis exacerbation. Platelet dysfunction poses a challenge to regional anaesthesia techniques, and haemostasis throughout an operation. Subtype Ib is caused by deficiency of glucose-6-phosphatase-translocase and is accompanied by neutrophil dysfunction, recurrent infections, autoimmune thyreoid disease, and inflammatory bowel disease.