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Cobb syndrome is a rare condition that includes a cutaneous nevus with an associated spinal vascular lesion at the same dermatome. We present a challenging case of a progressively symptomatic massive lumbosacral arteriovenous malformation (AVM) in a patient with Cobb's syndrome requiring a multimodality approach including staged preoperative transarterial and transvenous endovascular embolization followed by surgical excision and wound reconstruction.The patient presented with a massive lumbosacral AVM with symptoms of congestive heart failure. Angiography demonstrated arterial feeders predominantly from internal iliac, median sacral and lumbar segmental arteries.The patient underwent staged transarterial and transvenous endovascular embolization resulting in 90% reduction in the AVM size, followed by surgical resection of the lesion. The patient made an excellent recovery with improvement in his symptoms of congestive heart failure.The treatment algorithm for massive AVMs must be individualized. A combination of staged embolization and subsequent surgery may be required to obtain a good result. Through this carefully planned multidisciplinary approach a previously incurable lesion in this patient with Cobb syndrome was able to be treated successfully.