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Of 579 patients with intracranial dural arteriovenous fistulas, 545 had 1 fistula and 34 (5.9%) had enlarging, de novo, multiple, or recurrent fistulas. Of those 34 patients, 19 had progressive dural arteriovenous fistulas with de novo fistulas or fistula enlargement. Angioarchitectural correlates to chronically elevated intracranial venous pressures, such as venous sinus dilation and pseudophlebitic cortical venous pattern, were more common in progressive disease. Three young patients died despite endovascular, surgical, and radiosurgical treatments.