To report the case of bilateral congenital venous tortuosity and dilatation combined with cilioretinal artery. A 48-year-old woman complained of headache and underwent routine ophthalmic examination. The best-corrected visual acuity was 20/20 in both eyes. The examination of the fundus through a dilated pupil revealed that the retinal veins were strikingly tortuous and slightly dilated in the posterior pole and mid-peripheral retina of both eyes. However, the course and shape of the veins were normally straight in the peripheral retina. There was no change in the appearance and shape of the vein. The visual acuity remained unaffected during a 2-year follow-up.