Moyamoya disease is a cerebrovascular disease characterized by chronic and progressive occlusion of the distal internal carotid artery. As the disease progresses, it often leads to ischemic or hemorrhagic stroke. The incidence of moyamoya disease is high in East Asia, with cases occurring in both children and adults. It is a relatively common cause of pediatric stroke. Moyamoya disease can be diagnosed through specific imaging findings on cerebral angiography. Effective pharmacological interventions to reverse or decelerate the progression of the disease are currently lacking. The primary treatment option for moyamoya disease is revascularization surgery, which significantly reduces the risk of stroke in patients. Previous studies mainly focused on the clinical aspects and treatments of moyamoya disease in adults. However, children with moyamoya disease exhibit differences from adults in clinical manifestations, surgical treatment strategies, and perioperative management. This review summarizes relevant literature on the epidemiology, etiology, clinical manifestations, and treatment approaches of pediatric moyamoya disease.