A congenital retinal macrovessel (CRM) is a large retinal vessel, usually a vein, which traverses through the central macula and has large tributaries extending on both sides of the horizontal raphe. In the majority of cases, CRM have no effect on visual acuity, although in rare cases, macular hemorrhage, foveolar cysts, serous macular detachment, and the presence of the anomalous vessel in the foveola can affect vision. We describe a case of CRM with decreased vision secondary to a branch retinal artery occlusion (BRAO). To the best of our knowledge, this association has not been reported previously.