To evaluate the disease involvement in a patient with acute zonal occult outer retinopathy (AZOOR). Observational case report. A patient with acute zonal occult outer retinopathy was imaged with fundus photography, fluorescein and indocyanine green angiography, and autofluorescence photography. There was subtle depigmentation in the central portion of the lesion with a drusen-like deposit at the outer border. Fluorescein angiography showed a transmission defect centrally and a blocking defect at the border where the drusenoid material accumulated. Autofluorescent photography demonstrated that the drusenoid material was intensely autofluorescent, consistent with the presence of lipofuscin, and the central portion of the lesion showed atrophy of the retinal pigment epithelium. Indocyanine green angiography showed atrophy of the choriocapillaris underlying areas of atrophy of the retinal pigment epithelium. In this case acute zonal occult outer retinopathy caused an area of retinal pigment epithelium cell death with lipofuscin-laden cells at the border of the expanding lesion and associated atrophy of the underlying choriocapillaris.