To summarize the perioperative nursing experience of a child with liver-brain-type mitochondrial DNA depletion syndrome undergoing liver transplantation. Given the child’s energy synthesis abnormalities and metabolic disorders，the risk of metabolic storm before surgery，instability in hemodynamics and increased risk of hypothermia during the operation，as well as complex and diverse postoperative complications，the following measures were taken. A reasonable fasting strategy was developed preoperatively. Monitoring of hemodynamics was enhanced and hypothermia prevention measures were implemented intraoperatively. Postoperatively，a low-dose titration method for medication was adopted to accurately adjust drug doses and fluid strategy was dynamically adjusted to manage refractory ascites，and the individualized follow-up was conducted. After careful treatment and nursing，the child was discharged on the 38th day after the liver transplantation. After 6 months of outpatient follow-up，he recovered well.