Pseudoxanthoma elasticum in a 20-year-old man was complicated by right-sided proptosis, conjunctival vascular dilation, and subretinal bleeding from preexisting angioid streaks. Angiography showed multiple anomalies of major systemic arteries, including bilateral carotid rete mirabile and right carotid-cavernous sinus fistula. Electrothrombosis of the right cavernous sinus obliterated the fistula, restored cerebral perfusion by the right carotid system, and promoted prompt resolution of the presenting signs.