We studied 50 eyes of 44 patients with acute retinal necrosis, Kirisawa type uveitis (KU), in order to examine clinical symptoms, pathogenic viruses, clinical grading, therapy and prognosis for this disease. Varicella-zoster virus (VZV) was the pathogenic organism in 37 eyes of 31 patients, while herpes simplex virus (HSV) was responsible in 13 eyes of 13 patients. There were more elderly patients in the VZV-KU group than in the HSV-KU group. In addition, mutton fat keratic precipitates and retinal exudates were more common in VZV-KU than in HSV-KU. We divided KU eyes into 3 clinical grades: severe, serious, and mild. Using statistical analysis, we found that the VZV-KU group had a significantly greater number of severe and serious cases than the HSV-KU group. Furthermore, some HLA antigens were found to be statistically more common in the VZV-KU group, although no associations were found in the HSV-KU group. 32% of VZV-KU and 67% of HSV-KU eyes had a final visual acuity (fVA) of greater than 0.5. When eyes with an fVA of greater than 0.1 were compared to eyes with an fVA of less than 0.1, we found that combined therapy using acyclovir, interferon beta, and prednisolone was especially effective for VZV-KU, although no significant difference was found for HSV-KU. Thus, it is essential to determine the pathogenic virus causing KU, in order to understand the disease pathogenesis as well as to select appropriate treatment.