Childhood intracranial varix is rare and has been associated mostly with vein of Galen fistula or arteriovenous malformation. We present one patient with intracranial arteriovenous fistula with concomitant giant varix in a child. We treated the patient with endovascular embolization and obtained complete closure of fistula without morbility and mortality. The main radiologic and pathophysiologic features of this unusual entity, as well as the available therapeutic options, are discussed.