We report the case of a 6-year-old boy with Sturge-Weber syndrome and unilateral glaucoma in his left eye. He was born with a port wine mark involving his upper left eyelid. On ultra-widefield fluorescein angiography, he was found to have several vein-to-vein anastomoses in his left retina. To our knowledge, this is the first documentation of retinal vein-to-vein anastomoses in Sturge-Weber syndrome.