A 57-year-old woman with cutaneous manifestations of (BRBNS) presented with acute left proptosis and venous congestion secondary to thrombosis within a superior ophthalmic vein varix. Multiple phleboliths were noted in the contralateral right orbit, and an incidental right middle cerebral artery aneurysm. Her symptoms spontaneously resolved within a few days. An English literature review found 7 reported cases of orbital vascular lesions in association with BRBNS from 1950 to 2012. All lesions showed contrast enhancement on CT or MRI: 4 had small orbital calcifications and 3 were distensible with raised venous pressure. The occurrence of a thrombosed orbital varix or cerebral artery aneurysm in BRBNS, to the best of the authors' knowledge, has not been previously reported. Although orbital vascular lesions in BRBNS have been described as hemangiomas, the biologic behavior and histology of most of the reported orbital lesions are most compatible with venous malformations.