PURPOSE: Moyamoya disease is characterized by progressive narrowing of bilateral internal carotid arteries. Neuropsychological impairments are suspected due to frequent involvement of the frontotemporal areas. The present study thus aimed to investigate the pattern of neuropsychological function in children diagnosed with moyamoya disease. METHODS: Thirteen children with moyamoya disease of the transient ischemic attack type received standardized neuropsychological tests that evaluate general intellectual function, verbal omprehension, perceptual organization, working memory, processing speed, episodic memory, category fluency and visuospatial function. Related clinical factors were also analyzed.