We describe a case of bilateral exudative retinal detachment associated with prodromal symptoms simulating the presentation of acute Vogt-Koyanagi-Harada disease that was eventually diagnosed as acute lymphoblastic leukemia. A 42-year-old man presented with sudden visual loss in both eyes for two weeks. He complained of intermittent headache, neck stiffness and tinnitus for a month. His best-corrected visual acuities were 20/200 in both eyes. Fluorescein angiography, optical coherence topography and indocyanine green angiography featured bilateral serous retinal detachments. A clinical diagnosis of incomplete type Vogt-Koyanagi-Harada disease was considered. However, complete blood cell count showed a marked increase in the number of white blood cells and bone marrow examination revealed precursor B cell lymphoblastic leukemia. The patient started on induction chemotherapy. A week later, his best-corrected visual acuities were 20/25 and the serous retinal detachments were nearly absorbed in both eyes. Bilateral exudative retinal detachment associated with neurologic and auditory abnormalities may be a presenting sign of acute lymphoblastic leukemia. Clinicians should be aware of the possibility of leukemia in such patients. Korean J Ophthalmol 2009;23:325-328 ⓒ 2009 by the Korean Ophthalmological Society.