A 24-year-old woman with symptoms of chronic, recurrent headache, presumably secondary to migrainous etiology, came to our clinic and was found to have a congenital retinal macrovessel encircling an area superior to (but not passing through) the macula.Congenital retinal macrovessels are rare, aberrantly large branches of retinal arteries or veins that typically cross the horizontal raphe to either supply or drain the macular area. These unusual vascular entities must be distinguished from prepapillary vascular loops, racemose hemangiomas, Wyburn-Mason syndrome, or other arteriovenous malformations with potential neuro-ocular sequelae.