摘要Objective: To explore the perioperative nursing of a newborn with Beckwith-Wiedemann syndrome. Methods: We observed the blood glucose of the child and prevent infection of omphalocele before transportation and before the operation; after the operation, we performed airway nursing, blood glucose monitoring, urination observation, and tumor marker monitoring; finally, at the time of discharge from the hospital, we provided health education. Results: After a series of perioperative nursing measures, the child was discharged smoothly from hospital 34d after operation. Conclusions: Beckwith-Wiedemann syndrome is a rare congenital malformation. Timely and accurate observation and evaluation of the child during perioperative period and targeted nursing measures can effectively reduce or avoid the occurrence of postoperative complications.