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Background: Spinal dural arteriovenous fistulae are the most common type of spinal vascular malformations. They are presumed to be acquired anomalies, creating a direct communication between the radicular artery and the draining vein.Case Report: We describe a patient with rapidly progressive paraplegia who was misdiagnosed and treated as Guillain-Barré Syndrome. We discuss the clinical and imaging findings and the significance of magnetic resonance imaging in establishing the diagnosis.Conclusions: Although rare, this entity represents a potentially treatable cause of a progressive ischemic myelopathy and therefore, it is prudent that they are diagnosed and treated aggressively. The treatment alternatives include surgical obliteration or endovascular embolization of the fistula.