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 compatible with subretinal fluid and multiple pinpoint hyper- and hypofluorescent dots surrounding the detached retina. After 4 days, we were informed that the patient had been admitted to a general hospital due to spontaneous ecchymosis and melena. On complete blood count, there was a high white cell count, thrombocytopenia, and low hemoglobin concentration with a probable diagnosis of leukemia. On bone marrow biopsy, ALL with B-cell precursor was confirmed. In conclusion, exudative retinal detachment can be a primary presentation of leukemia and/or lymphoma. In atypical VKH or acute central serous chorioretinopathy cases with multiple pinpoint leakages distributed mostly in the periphery of the subretinal fluid area (not randomly scattered throughout the subretinal fluid), we should consider leukemia.