Purpose: We describe a central retinal artery occlusion with cilioretinal sparing in a young male patient who was found to have mitral valve papillary fibroelastoma.
Methods: At the initial examination, a 33-year-old Hispanic man had visual acuity of 20/200 in his left eye, and 2 weeks later, visual acuity improved to 20/20. Diagnosis required transesophageal echocardiography to localize the lesion.
Results: Mitral valve papillary fibroelastoma involving the mitral valve was successfully treated with tumor resection.
Conclusion: Routine echocardiography should be performed in all patients presenting with central retinal artery occlusion as it may diagnose treatable cardiogenic etiologies and present further potentially life-threatening embolic events.