Background: Dural arteriovenous fistula (dAVF) rarely presents with a syndrome of reversible parkinsonism and rapidly progressive dementia, which has been described in 19 patients to date. However, its presenting features, pathophysiology, and response to treatment have not been reviewed. Methods: We report the clinical course and treatment of two novel patients with this syndrome. Results: Despite severe presentations, both patients recovered fully after fistula obliteration. Conclusions: Atypical parkinsonism with early cognitive changes and other focal neurological signs, along with features such as pulsatile tinnitus or worsening headache, should raise the possibility of dAVF. When treated, this syndrome is nearly always reversible, highlighting the importance of early diagnosis.Dural arteriovenous fistulae (dAVF) are pathologic shunts between dural-based arteries and dural venous sinuses, meningeal veins, or cortical veins. Common presenting symptoms include pulsatile tinnitus and mastoid bruit in transverse-sigmoid fistulae, and proptosis, visual deterioration, chemosis, ophthalmoplegia, and orbital bruit in cavernous sinus fistulae. 1