Clinical features of infective endocarditis (IE) may come from systemic infections, intravascular lesions, immunological responses, and its complications. It has varied manifestations which present to emergency department physicians. This report describes a case of IE in a 38-year-old man who had fever, headache, abdomen pain, and notably, acute blindness at the beginning of the illness. The blindness was secondary to embolic infarction of both occipital lobes developing as a sequela of IE. This is an unusual neurologic complication of IE, tends to occur in the early stage of the illness, and may be related to Staphylococcus aureus infection. To reduce mortality and complications, early diagnosis and treatment are critical. This case indicates that IE should be considered in cases of acute cortical blindness, especially those with fever and heart murmur.