Glycogen accumulation disease, due to impaired glycogen breakdown, is often prone to severe hypoglycemia and acidosis in children with fever, diarrhea, vomiting, and inability to eat normally, which can manifest as pallor, sweating, irritability, and in severe cases, convulsions, unconsciousness, shortness of breath, and even coma and death. It requires emergency treatment by the patient’s parents or even by the hospital. Parents should acquire knowledge of the routine emergency management of this disease and inform the emergency physician of their child’s condition. 1. Hypoglycemia: Plasma blood glucose concentration <2.8 mmol/L, or whole blood glucose <2.5 mmol/L. 2. Clinical manifestations: The child may show convulsions, unconsciousness and rapid pulse; those without convulsions may show panic, hunger, pale face, sweating and rapid heart rate. At this time, the emergency blood glucose is often <2.8 mmol/L. 3. Other precautions: When the child has infection, additional glucose supplementation is often needed, especially when fever, vomiting and diarrhea are present, and the amount of glucose can be given according to the maintenance period in the above table. When surgical procedures require fasting, intravenous glucose supplementation and blood glucose testing are required.