Objective To explore the feasibility of perioperative management of ischemic moyamoya disease in children and to discuss the effect of perioperative ischemic events on clinical prognosis. Methods Retrospectively analyzed the clinical data and follow-up data of 28 children with 36 sides operation with ischemic moyamoya disease from May 2011 to December 2017 in our hospital. In view of the comprehensive improvement of perioperative management plan after June 2015, the patients were divided into A group and improved group. The difference of perioperative ischemic events between the two groups was evaluated, and the clinical symptoms were evaluated at 6 months after operation. Results The difference of perioperative ischemic events between group A (4/10) and group B (2/26) was statistically significant (P<0.05). At 6 months after operation, the clinical symptoms of 86.11% (31/36) improved There were 5 cases with no significant change or deteriorate of clinical symptoms, including 4 cases had a history of ischemic events during perioperative period, which accounted for 80% of the total (4/5). Only 33.3% (2/6) of the patients had surgical side prognosis and clinical symptoms improved, which have occurred perioperative ischemic events. Conclusions Through comprehensive improvement of perioperative management plan, we can effectively reduce perioperative ischemic events in children with ischemic moyamoya disease. Perioperative ischemic events may be the main reason for no significant changes or deteriorate of clinical symptoms.