Background: Central retinal Artery occlusion (CRAO) is an ophthalmic emergency and the ocular analogue of the cerebral stroke. Atrial myxomas are the most common benign primary cardiac tumor. We report a case of undiagnosed left atrium (LA) myxoma who presented with sudden onset blindness in right eye due to CRAO as a sole manifestation and echocardiographic characteristics of myxoma which increases the risk of embolism. Methods: A 52-year old woman presented with history of sudden onset blindness in right eye. Fundus examination was suggestive of CRAO. Transthoracic and transesophageal echocardiogram showed a mass in LA compatible with LA myxoma. Complete surgical resection of myxoma was done although vision could not be restored to normal. Conclusion: Detailed history and complete systemic examination should be done in every patient with embolic phenomena and early neuroimaging with echocardiography using newer modality like 3D imaging should be used even in absence of electrocardiographic or auscultatory abnormalities. Key words: Myxoma, retinal artery occlusion, embolism, 3 dimensional echocardiography