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A case of spinal dural arteriovenous fistula arising from a branch of the internal iliac artery is reported.To report a rare case of spinal dural arteriovenous fistula supplied by a lateral sacral artery and treated with endovascular therapy.Spinal dural arteriovenous fistulas usually occur in the thoracic and lumbosacral regions and arise from the intercostal and lumbar arteries. Rarely, they may occur in the sacral region, as in the reported case.A 60-year-old man presented with progressive lower extremity paresis and decreased sensation below the waist of 6 months duration, which had progressed to paraparesis. Diagnostic imaging included magnetic resonance imaging and spinal angiography.A sacral spinal dural arteriovenous fistula was diagnosed with spinal angiography, which showed the spinal dural arteriovenous fistula arising from the right lateral sacral artery branches at S2, and magnetic resonance imaging, which showed enlarged pial vessels along the surface of the spinal cord and central cord hyperintensity, with peripheral hypointensity on T2-weighted images. The patient was definitively treated with endovascular therapy using polyvinyl alcohol particles and Tornado coils. His symptoms almost completely resolved within 6 months of therapy.Although surgical ligation is the treatment of choice, endovascular therapy may be an effective treatment for patients with sacral region spinal dural arteriovenous fistula in cases of high surgical risk. Spinal angiography remains the definitive diagnostic examination for pinpointing the site of the dural arteriovenous fistula.