Moyamoya disease (MD) is a cerebrovascular pathology. Characterized by a stenosis of the terminal segments of the internal carotid artery. Currently the etiology of MD isn’t clear. Different studies have focused their research on understanding the disease from epidemiological aspects, its pathophysiology and recently the neurocognitive alterations presented by patients. This disease has a progressive course that impacts cognitive function. To identify the neurocognitive phenotype presented by a patient diagnosed with MD. He’s 9-year-old male patient with sudden deterioration of his neurological status. With motor aphasia, right hemiplegia and increased tone of his left hemibody. An evaluation protocol was applied by clinical neuropsychology with general and specific tests aimed at assessing the neurocognitive functioning of the patient. The patient presents alterations in information registration tasks, sustained attention, visuospatial functioning and visuoconstructive skills, compatible with dysexecutive syndrome.

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