Purpose: This report describes a case of amaurosis fugax due to a rare primary cardiac sarcoma. Observations: A patient who was recently diagnosed with left atrial pleomorphic sarcoma presented with a chief complaint of multiple episodes of intermittent vision loss in the right eye during the course of radiation therapy. Conclusions and importance: The authors postulate emboli from the left atrial sarcoma entered systemic circulation and subsequently caused brief episodes of transient occlusion to retinal, ophthalmic and/or ciliary arteries leading to momentary retinal hypoxia. We believe this is a novel finding, previously unreported in the literature, of transient embolic occlusion without permanent visual sequelae due to a malignant primary cardiac pleomorphic sarcoma.