Multiple evanescent white dot syndrome (MEWDS) is an acute retinal disease characterized by multifocal white spots in the fundus often seen in the unilateral eye. The lesions mainly involve the retinal pigment epithelium and the outer retinal structure. Typical ocular manifestations of MEWDS include grayish-white outer retinal spots with a clear borderline identified on the fundus, findings of hyper-autofluorescence in the early stage consistent with the spots identified on the fundus, and the optical coherence tomography manifestation of multifocal disruption of the ellipsoid zone. With the rapid development of multimodal imaging technology, some scholars found that these manifestations are not exclusive to MEWDS as some types of chorioretinopathy can also show MEWDS-like changes. The etiology of these diseases may be inflammation, infection, immunity, or tumor-related, misdiagnosed by masquerading as MEWDS. Here we summarized the clinical manifestations and imaging features of MEWDS and reviewed the fundus lesions changes that can be misdiagnosed as MEWDS.