Perifoveal exudative vascular anomalous complex (PEVAC) are class of diseases characterized by isolated aneurysmal lesions of the perifovea with hemorrhage and hard exudates, which are found in recent years. Its pathogenesis and pathological process are not yet fully elucidated. The current consensus through multimodal imaging studies is that PEVAC is an idiopathic perifoveal retinal vascular abnormality with cystoid macular edema (CME) and hard exudate. It is not easily distinguished clinically from retinal microaneurysm due to diabetic retinopathy or retinal vein occlusion, aneurysmal telangiectasia, and type 3 macular neovascularization (stage Ⅰ). PEVAC is a partially self-healing property, and some aneurysms and CME may resolve on their own during follow-up period. Laser photocoagulation is an effective measure for the treatment of PEVAC. In the future, with the rapid development of imaging technology, the progress of research on pathogenesis and treatment strategies and the updating of theories are expected to provide more effective treatments for PEVAC.