OBJECTIVEPediatric pial arteriovenous fistulae (pAVF) are rare vascular lesions of the CNS, reported to have up to a 25% association with hereditary hemorrhagic telangiectasia. The presentation, treatment and syndromes associated with pAVF in children are reported here.DESIGNA pediatric database for pAVF was retrospectively reviewed. Patients with carotid-cavernous fistulae, dural arteriovenous fistulae, brain arteriovenous malformations and vein of Galen malformations were excluded. Radiographic outcome was assessed using digital subtraction angiography, and clinical outcome by the Functional Status Scale (6=normal, maximal incapacity=30).RESULTSBetween July 2003 and June 2011, seven patients with pAVF (six intracranial and one spinal) were treated. Mean age was 4.2 years. The most common clinical presentation was high output cardiac failure (43%). Two patients (29%) harbored a known mutation in the RASA1 gene, associated with a hereditary vascular syndrome: capillary malformation-arteriovenous malformation. No patient had hereditary hemorrhagic telangiectasia. Treatment resulted in complete lesional obliteration in six of seven patients, with treatment ongoing in the seventh. Five of seven patients had combined endovascular and surgical treatment while two underwent endovascular embolization alone. Functional Status Scale scores at the most recent follow-up were 6 in all but one patient who had presented with a pretreatment hemianopsia.CONCLUSIONTreatment is effective in obliterating pAVF in children, with an excellent prognosis seen in our cohort. Genetic screening is indicated, with capillary malformation-arteriovenous malformation being the most frequently seen syndrome. No patients had hemorrhagic hereditary telangiectasia.