Glycogen storage disease type I is a rare autosomal recessive inherited disorder with an annual incidence of approximately 1:100,000 . Due to a deficiency of glucose-6-phosphatase , 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  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 , and platelet dysfunctions  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.