An intracranial varix is rare and has been associated mostly with vein of Galen fistulae or arteriovenous (AV) malformations. The authors present eight cases of intracranial, pial or subpial AV fistulae with concomitant giant varices. Six were supratentorial and two were infratentorial. Only one case involved the vein of Galen. In six cases successful surgical and/or endovascular occlusion of the intracranial AV fistula was obtained, and one case was treated conservatively. Staging of surgery and postoperative hypotension were considered to be important in avoiding edema and hemorrhage following obliteration of a large AV shunt. One patient died from delayed postoperative intracerebral bleeding.