To summarize the nursing experience of a patient with glycogen storage disease complicated with sepsis and cold agglutination phenomenon. Glycogen storage disease is a rare metabolic disorder，factors such as fever and infection can easily induce acute metabolic crisis，and the patient was accompanied by cold agglutination，its treatment and nursing were complicated. The key points of nursing care included blood sugar and nutrition management，acidosis correction，infection control，evidence-based target body temperature management，ensuring safe blood transfusion. After 8 days of active treatment and careful nursing care，the patient’s condition improved and she was discharged. After 6 months of follow-up，she returned to the hospital as scheduled，the recovery was good.