A high flow pial arteriovenous fistula in a twenty-years-old girl is described. The arteriovenous communication corresponds to a single hole fistula on the right rolandic area, with a giant venous ectasia. The patient presented seizures and left hemiparesis as symptoms. The fistula was embolized with glue obtaining total occlusion of the shunt. Hypotension was induced and valsalva manoeuver was done during the glue injection to reduce the flow into the fistula, however 26 hours after the procedure the patient bled resulting in a fatal outcome. The purpose of this paper is discuss the presentation of Rendu-Osler-Weber (ROW) in children and the therapeutic guidelines.