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We report a 7-year-old boy with Menkes disease complicated by rupture of a large splenic artery aneurysm. The aneurysm was successfully embolized with microcoils and n-butyl cyanoacrylate. Further angiographic evaluation revealed marked tortuosity of mesenteric and lower extremity vasculature, including the femoral arteries bilaterally, without aneurysm formation. The patient has since been evaluated annually with computed tomography angiography and there have been no additional vascular complications of his disease during 3-year follow up. Keywords: Aneurysm, Embolization, Menkes