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Acute zonal occult outer retinopathy (AZOOR) is a relatively rare ocular disease that is characterized by the rapid loss of one or more zones of outer retinal function. AZOOR usually occurs in young women. AZOOR patients typically present with the sudden onset of photopsia and acute scotomas related to loss of sectors of outer retinal function. Early in the course of AZOOR, funduscopic appearance was often normal; however, most patients developed zones of retinal pigment epithelial atrophy or pigment clumping over time. Changes in electroretinogram (ERG) and persistent visual field defects were usually observed. Optical coherence tomography (OCT) showed that inner segment (IS)/outer segment (OS) regional abnormal changes corresponding to the visual field defect location area, at the same time the morphology and position of the visual field defect and multifocal electroretinogram (mfERG) shown in retinal amplitude density decreased regions coincide. The visual function of some patients can permanently damage. Fundus examination, mfERG and fluorescein angiography (FA), indocyanine green angiography (ICGA), autofluorescence (AF) and frequency domain OCT imaging techniques has an important significance for the diagnosis of AZOOR. Ever since the lack of understanding, AZOOR is often missed or misdiagnosed. In order to improve the understanding of the disease, this paper reviewed the definition, etiology, the epidemiological characteristics, clinical manifestation, classification, diagnosis, differential diagnosis, treatment and prognosis of AZOOR.