Of the 185 carotid and vertebral fistulas treated by the authors over the past 10 years, five developed neurologic deficits after abrupt closure of their fistulas. The earliest case, treated initially by proximal surgical carotid occlusion, presented 32 years later with cerebral steal symptoms from the large, long-standing carotid cavernous fistula. Upon completion of a surgical trapping procedure, there was immediate massive cerebral edema, brain herniation, and death. In the remaining four patients (three vertebral fistulas and one with carotid cavernous fistula), all treated by transvascular embolization techniques, neurologic deficits occurred coincidentally with the abrupt closure of the fistula and resolved with reestablishment of fistula flow. This indicated that the cerebral vasculature is unable to tolerate the reestablishment of normal cerebral perfusion after abrupt closure of the fistula. All four patients were treated with staged or slow occlusion of their fistulas, which resulted in complete fistula closure without permanent neurologic sequelae. All five patients who developed symptoms consistent with normal perfusion pressure breakthrough had large, long-standing fistulas, ranging in duration from 9 to 32 years. Two of the five patients developed slowly progressive neurologic deficits consistent with cerebral steal prior to treatment. This sign was not observed in the 180 patients who did not develop symptoms during treatment. We conclude that patients with carotid or vertebral fistulas of long duration, particularly those with cerebral steal symptoms, are at risk to develop neurologic deficits related to perfusion breakthrough if their fistulas are abruptly closed. Staged or gradual closure may prevent this potentially devastating complication.