Abstract:【】 Cognitive impairment in Moyamoya disease, classified as non-dementia vascular cognitive impairment, demonstrates fundamental differences from neurodegenerative disorders such as Alzheimer"s disease, Huntington"s disease, and frontotemporal dementia across multiple domains including pathogenesis, pathophysiological alterations, clinical manifestations, and diagnostic/therapeutic principles. Current research on this complication remains fragmented with conflicting findings. This review synthesizes existing evidence regarding epidemiology, mechanistic pathways, neuroanatomical targets, clinical presentations, diagnostic approaches, and therapeutic interventions for Moyamoya-related cognitive decline, while proposing future research directions. The synthesis aims to enhance clinical recognition of this underinvestigated complication, emphasizing early identification and timely intervention. We hypothesize that proactive management may delay the progression of cognitive decline and improve long-term patient outcomes through optimized cerebrovascular perfusion and neuroprotective strategies.