During the 12 years from 1977 to 1988 in Tottori University Hospital, 6 cases (4.2%) with chronic active "lupoid" hepatitis were encountered among 143 patients with histologically proven chronic active hepatitis. HLA antigens were studied; BW 22 and CW 1 were strongly associated, and A 11 was rather associated with the rare disorder in Japan. These results suggested that Japanese patients with chronic active "lupoid" hepatitis have different HLA markers from those in Caucasian patients, which may be attributable to rare specificity in the HLA system rather than different mechanisms of pathogenesis.