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Introduction: Moyamoya disease is a rare form of the chronic cerebrovascular disorder. Patients develop progressive stenosis or occlusion of the terminal portion of the internal carotid arteries and a proximal portion of the anterior and middle cerebral arteries. The compensatory collateral circulation that develops is weak and small, prone to hemorrhage, aneurysm, and thrombosis. Case Report: We report a case of a six-year-old boy with Moyamoya disease, presenting with complaints of seizures and weakness of the left side of the body. A bilateral encephalo-myo-synangiosis (EMS) was successfully performed on the child under general anesthesia without any perioperative complications. Conclusion: Revascularization surgery in a patient with Moyamoya disease is a high-risk procedure with an increased incidence of perioperative neurological sequelae. However, patients can be successfully perioperatively managed by ensuring adequate cerebral blood flow and minimizing cerebral metabolic oxygen consumption rates.