A man in his mid-50s with previously untreated HIV 1 infection presented with bilateral, violaceous, caruncular lesions. Examination of the left eye showed a violaceous, firm, pedunculated mass protruding nearly 10 mm, extending into the adjacent conjunctiva, and additional eyelid lesions with mild edema (Figure, A). Pathologic examination of similar-appearing oral mucosa lesions showed vascular and spindle cell proliferation with fascicles of atypical spindle cells that stained strongly for human herpesvirus 8, CD31, and erythroblast transformation specific–related gene, confirming a diagnosis of Kaposi sarcoma.1 Further evaluation revealed a CD4 helper T-cell count of 13 cells/μL and bilateral lung and possible liver involvement. The patient started highly active antiretroviral therapy (HAART) and liposomal doxorubicin treatment given the presence of concurrent systemic disease. Nearly 8 months later, examination showed complete regression of bilateral ocular lesions (Figure, B). Ocular Kaposi sarcoma is rarely described as an AIDS-defining illness.2,3 Importantly, active eye disease may be associated with systemic disease.