OBJECTIVE To report a unique case of pacemaker-related infective endocarditis manifesting as endogenous endophthalmitis with chorioretinitis secondary to Histoplasma capsulatum. METHODS Case report. RESULTS A 75-year-old man was diagnosed with blood culture-negative infective endocarditis and was admitted with deteriorating vision and ocular inflammation. Examination of the eye indicated significant vitreous inflammation and retinitis. Vitreous cultures were negative, but universal fungal polymerase chain reaction of the vitreous fluid was positive for H. capsulatum. Histopathology of the fibrous cuff around the extracted right atrial lead demonstrated hyphal and yeast forms and polymerase chain reaction of this material identified H. capsulatum. Despite aggressive antifungal and surgical treatment, the eye became phthisical. CONCLUSIONS We highlight the importance of considering H. capsulatum in the differential diagnosis of endogenous endophthalmitis, particularly among patients from endemic areas who present with possible endovascular infection.