A 39-year-old male, who was treated as Takayasu arteritis by immunosuppressive therapy (corticosteroid and cyclosporine A) for 2 months, presented to our hospital due to malaise, remittent fever (38°C), and repeated syncope episodes. His skin revealed a solitary painful erythematous nodular lesion on the right forefinger (Figure 1A, arrow) and some erythematous painless macules on the bilateral upper limbs (Figure 1B, arrows). Transesophageal echocardiography demonstrated mild mitral-valve regurgitation according to the prolapsing of posterior mitral leaflet with a high echoic mass on the anterior mitral leaflet (Figure 1C, arrow). 18F-fluorodeoxyglucose (FDG)-positron emission tomography (PET) demonstrated intense FDG uptake in the left ventricular myocardium, but no FDG uptake on the native valves or aortic wall (Figure 2). The ophthalmologic examination found bilateral white-centered retinal spots, suggesting Roth spots (Figure 3A, arrows). Peripheral blood culture repetitively identified Streptococcus oralis. The immunosuppressive therapy was discontinued and antimicrobial including penicillin G and gentamycin were initiated. Three weeks after admission, the Roth spots were invisible (Figure 3B). After the initiation of antimicrobial, the patient had a transient left-sided hemiparesis with multiple cerebral infarctions (Figure 3C). On the basis of his condition, a resection of the vegetation and patch valvulo-plasty were performed. After the surgical therapy, he was discharged without any damage of systemic complications. Among clinical hallmarks of infective endocarditis, Osler nodule, Janeway lesion, and Roth spot are essential features to identify. In this case, his retinal appearance was the most critical to diagnose infective endocarditis. Retinal examination is of prominent importance in the consideration of possible infective endocarditis.