Abstract
The glycogen storage disease type 1b is a rare metabolic disease caused by the glucose 6-phosphate transporter disorder. This study presents a 10-year-old female patient with growth retardation, neutropenia and inflammatory bowel disease, severe hypoglycemia attacks before and during the surgery, and hepatocellular carcinoma who underwent liver transplantation. A multidisciplinary approach from the preoperative preparation stage is essential in addition to intraoperative intervention for hypoglycemia and lactic acidosis in the anesthetic management of these patients.
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