Abstract: Moyamoya disease is a specific cerebrovascular disease characterized by progressive narrowing of the terminal part of the internal carotid artery and its major branches, and these changes lead to chronic cerebrovascular occlusion and the formation of an abnormal vascular network. Previous studies have found that the pathogenesis of moyamoya disease is related to genetics, inflammation, immune response and other factors, but its etiology and pathogenesis are still unclear. In recent years, great breakthroughs have been made in the study of the pathogenesis of moyamoya disease in the aspect of inflammation. Inflammatory factors and inflammationrelated proteins play an important role in the pathogenesis of moyamoya disease, and the inflammatory response mediated by autoimmune diseases can also promote the pathogenesis of moyamoya disease. However, although some progress has been made in the study of the relationship between inflammation and moyamoya disease, no systematic framework has been formed for the mechanism of how inflammation specifically promotes the development of moyamoya disease, and relevant studies are still in the stage of scattered exploration. Most of the existing literatures focus on single inflammatory factors or local pathological processes, and lack a comprehensive analysis of the dynamic role of inflammation in the whole course of moyamoya disease. By searching the existing literature on the role of inflammation on the pathogenesis of moyamoya disease in the database, and comprehensively summarizing the literature, this review aims to deeply understand the influence of inflammatory factors on the pathogenesis of moyamoya disease, and then provide theoretical support and practical guidance for the comprehensive treatment strategy of moyamoya disease.