A 55-year-old woman with no known systemic disorder and without any history of ocular disease, trauma, and surgery presented with a nonremitting conjunctival redness on her left eye that was existing since her childhood. On ophthalmological examination, an extremely rare coexistence of isolated unilateral bulbar conjunctival telangiectasia and ipsilateral retinal vascular tortuosity without any systemic and neuroradiological association was detected. We aimed to demonstrate this rare vascular coexistence and discuss differential diagnosis of the underlying causes.