Pial arteriovenous fistula (AVFs) are a rare, although clinically significant, vascular anomaly affecting the pediatric population, consisting of a high-flow connection between one or more pial arteries and the venous. In children, the clinical presentation varies according to age, can present with high output cardiac failure, macrocrania, neurodevelopmental delay, seizures, venous infarctions due to venous stenosis or hemorrhage secondary to venous thrombosis. The treatment, is a multimodal approach, most commonly endovascular, is effective in the obliteration of AVF. A case is presented of a previously healthy infant boy with neurological deficit arising from pAVF of the middle and posterior cerebral artery. The pAVF was successfully treated with endovascular occlusion.