Human T-cell lymphotropic virus type I (HTLV-I) has been reported as the cause of a kind of endogenous uveitis (HTLV-I associated uveitis; HAU). We observed a case of episcleritis in a HTLV-I carrier with pigmentary retinal degeneration. HTLV-I infection might be associated with the development of episcleritis and pigmentary retinal degeneration. Patients with episcleritis or pigmentary retinal degeneration should be examined for HTLV-I infection.