• Acute posterior multifocal placoid pigment epitheliopathy is a chorioretinal inflammatory disease occurring in young, healthy adults. Its cause is unknown, although it frequently follows a flulike illness. We reexamined 30 patients with documented acute posterior multifocal placoid pigment epitheliopathy to determine their HLA class I antigen (A and B) and class II antigen (DR and DQ) distribution. The HLA class I antigen B7 was found in 12 patients (40.0%) compared with 63 controls (16.6%) (relative risk, 3.38). The class II antigen DR2 was present in 17 patients (56.7%) compared with 107 controls (28.2%) (relative risk, 3.34). The specific role of HLA antigens in uveitis is unknown, but the finding of an increased prevalence of HLA-B7 and HLA-DR2 antigens in patients with acute posterior multifocal placoid pigment epitheliopathy suggests an immunogenetic predisposition to acquiring this disease.