In this report, we detail a rare presentation of central retinal vein occlusion (CRVO) in a patient with fibromuscular dysplasia (FMD). A 45-year-old woman with a 12-year history of FMD presented to the ophthalmology clinic with symptoms and exam findings consistent with CRVO. Dilated fundus examination revealed disc edema, diffuse flame, and dot-blot hemorrhages, and tortuous, engorged retinal veins. The patient was diagnosed with CRVO, and she was treated with monthly anti-VEGF monoclonal antibody followed by a VEGF inhibitor. At her most recent follow-up, her macular edema was resolved and her visual acuity had markedly improved. FMD has been shown to rarely present with retinal manifestations, especially in patients with hypertension. This appears to be first case report to document CRVO in the context of known FMD. We suggest that CRVO be considered as a potential complication for young patients with FMD.