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We report a case of unilateral varicella-zoster virus (VZV) related keratouveitis and papillitis. A 54-year-old male presented with headache, left forehead rash, and decreased vision in the left eye. Examination of the left eye showed visual acuity (VA) of 0.15, relative afferent pupillary defect, anterior stromal corneal edema, nongranulomatous keratic precipitates, a swollen and hyperemic optic disc. The patient could read 6/12 of the Ishihara plates in the left eye. Optical coherence tomography (OCT) showed optic disc edema and OCT angiography (OCTA) revealed increased peripapillary vascularity. Computerized visual field testing demonstrated an enlarged blind spot and lower altitudinal defect. Serum anti-varicella-zoster IgM antibody level was elevated. The patient was put on oral acyclovir and prednisone treatment. At 1-month follow-up visit, VA increased to 0.9 and color vision was 11/12 Ishihara plates. OCT showed resolution of optic disc edema and OCTA demonstrated normalization of the increased peripapillary vascularity. Computerized visual field test demonstrated improvement in the lower altitudinal visual field defect and normalization of the enlarged blind spot. Optic nerve involvement in VZV infection occurs rarely, especially in immunocompetent patients as in our case.