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We aimed to describe a case of acute lymphoblastic leukemia (ALL) that initially presented and was managed as Vogt-Koyanagi-Harada syndrome (VKH). A 62-year-old man was referred for vision loss starting 1 week ago. There was no prior systemic or ocular history. Visual acuity was 20/200 in both eyes. Anterior segment exam was also normal in both eyes. On funduscopy, foveal reflex was significantly reduced. On optical coherence tomography, subretinal fluid was evident in both eyes. Fluorescein angiography showed a hypofluorescent area co