OBJECTIVE Recently HTLV-I has been shown to cause a kind of endogenous uveitis in south west Japan, where HTLV-I infection is highly endemic. To investigate further the association of HTLV-I infection with the incidence of this uveitis, HTLV-I seroprevalence in central Japan, where HTLV-I infection is not endemic, was studied. METHODS HTLV-I seroprevalence was investigated in 1579 patients with various ocular diseases and 1251 normal volunteers as a younger control group. Then HTLV-I seroprevalence was compared in each group. RESULTS Of 1579 patients with various ocular diseases, 38 (2.41%) were seropositive. There was a statistically significant difference in HTLV-I seroprevalence between the undefined uveitis group and non-uveitic ocular diseases group (p < 0.05, Yates's correction). However, the seroprevalence in younger patients with undefined uveitis did not differ significantly from that in other groups. As regards the incidence of this type of uveitis, six of 12 (50%) seropositive patients, who were born in south west Japan and had lived in this area for 35 years, developed this undefined uveitis whereas only two of 26 (7.69%) seropositive patients in the other areas in Japan developed this uveitis. The difference was statistically significant (p < 0.05, Fisher's exact probability test). CONCLUSIONS These results suggest that the incidence of this type of endogenous uveitis could be greatly influenced by environmental or hereditary factors including HLA.