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Foix-Alajouanine syndrome is a rare progressive form of spinal AVM predominantly affecting the lower thoracic and/or lumbosacral regions. This study aims to describe the imaging findings of spinal AVM causing Foix-Alajouanine syndrome and to review the literature. We present a 48-year-old man with progressive back pain, leg weakness, and gait imbalance without urinary retention. We discuss the clinical and imaging findings and the significance of MRI in establishing the diagnosis. A definitive diagnosis of spinal AVM requires radiographic demonstration of the vascular anomaly. Despite the high sensitivity of angiography for the diagnosis of spinal AVM, the result of the study may be inconclusive and/or negative. The key MRI findings are the presence of abnormally dilated perimedullary vessels with signal voids from a high-velocity flow on T1 and T2 weighted images.