This 1-year-old male infant had been diagnosed with cerebral degenerative disease because he developed psychomotor regression, and brain atrophy was demonstrated on computed tomography. He underwent magnetic resonance imaging, which suggested a cerebrovascular malformation. Cerebral angiography disclosed an arteriovenous fistula, fed by an anterior cerebral artery and directly draining into a cortical vein. The occlusion of the feeding artery was followed by a satisfactory recovery. The mechanism of the neurological symptoms in this patient is thought to be caused by a steal phenomenon and compression of the brain stem by venous engorgement in the posterior fossa.
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