We present two cases of mitral valve lesions that manifested with unilateral blindness caused by central retinal artery occlusion (CRAO): Case 1. A 68-year-old woman was admitted to our clinic for sudden blindness. Retinal artery angiogram showed CRAO. Transthoracic and transesophageal echocardiography (TEE) documented a mass attached to the ventricular side of the posterior mitral leaflet, which at pathology was identified as a blood cyst. Case 2. A 67-year-old man was admitted for a sudden unilateral painless loss of vision. Retinal angiogram documented CRAO, and TEE showed a highly mobile, spherical, lesion on the atrial side of anterior mitral leaflet. In this case, the pathological finding was a degenerated calcified thrombosis. We report on two cases of very rare abnormalities of the mitral valve presenting with a very rare embolic complication, i.e., CRAO. Like for cryptogenic stroke, transesophageal echocardiography plays a central role in the diagnosis of cardiogenic embolic sources.