Extracranial vertebral artery aneurysms represent an uncommon presentation of collagen vascular disease. We performed staged proximal embolization of large left vertebral artery aneurysm after distal common carotid-to-vertebral bypass at C2 in a young adult patient with Marfan syndrome and a hypoplastic contralateral vertebral artery. Dilation of the autogenous saphenous vein graft occurred at 1 year with proximal graft stenosis requiring operative revision. Subsequent dilation of the basilar artery led to symptoms of pontine compression at 18 months that have resolved at 31 months of follow-up.