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Purpose: To report a case of unilateral progressive atypical acute zonal outer retinitis in a healthy young female.Case summary: A 32-year-old healthy woman presented with visual disturbance in the left eye for 1 week. Best corrected visual acuity was 0.4 in the left eye. No abnormal findings were detected on brain magnetic resonance imaging and a laboratory work-up. Zonal retinal opacity around the optic nerve disc was noticed in a fundus photo which was consistent with the outer retinal signal defect on optical coherence tomography and a defect in the central visual field from perimetry. Multiple evanescent white dot syndrome was suspected based on fluorescein angiography and indocyanine green angiography. Prednisolone was prescribed. Visual acuity was reduced to hand-motion after 10 days with extended zonal retinal opacity. No virus was detected by multiplex polymerase chain reaction of the aqueous humor. The lesion did not improve despite high-dose intravenous steroid and antiviral treatment.Conclusions: Visual function was lost with the progression of outer retinitis. This case differed from previous cases as the outer retinitis progressed rapidly from the posterior pole to the peripheral retina. It was named fulminant progressive atypical acute zonal outer retinitis.

Fulminant Progressive Atypical Acute Zonal Occult Outer Retinitis