AIM: To comprehensively examine the clinical presentations, multimodal images, and long-term follow-up of Chinese patients with acute zonal occult outer retinopathy (AZOOR), a rare inflammatory disorder. METHODS: This was a retrospective study. A total of 20 patients (32 eyes) were included. The medical records and multimodal imaging, including wide-field fundus photography, wide-field fundus autofluorescence (FAF), and swept-source optical coherence tomography (SS-OCT) were analyzed. RESULTS: The study included 20 patients with a mean age of 38.2±10.9y, and females accounted for 60%. Lesions could involve peripapillary areas, macular region, and peripheral retina. The mean best-corrected visual acuity (BCVA) at presentation was 0.38±0.60 logMAR, with no significant difference in visual acuity between acute cases (within 6mo of onset) and chronic cases (beyond 6mo of onset; P=0.390). There was no statistically significant difference in visual acuity between eyes of acute case (within 6mo of onset) and the chronic case (beyond 6mo of onset). In some chronic case, FAF examination revealed the presence of a hyperautofluorescent (hyperAF) ring around the macular area (6/18), a phenomenon not observed in the acute case (P=0.024). A higher proportion of chronic cases showed predominantly hypoautofluorescent (hypoAF) lesions compared to the acute case (13/18 vs 2/14, P=0.0016). SS-OCT examination showed that both acute and chronic cases exhibited hyperreflective dots above the retinal pigment epithelium (RPE), and ellipsoid zone (EZ) and RPE damage. In the chronic case, eyes with hyperreflective dots above the RPE were more likely to exhibit EZ and RPE damage in the macular region compared to those without these dots. CONCLUSION: Multimodal imaging plays a crucial role in the follow-up of patients with AZOOR. In chronic cases of AZOOR, the presence of hyperreflective dots above the RPE indicates a higher likelihood of outer retinal involvement in the macular region. This study provides critical insights into the complex presentation and progression of AZOOR.