Perifoveal exudative vascular anomalous complex (PEVAC) is a very rare macular vascular disease characterized by an isolated large aneurysmal lesion in the fovea, with accompanied by small retinal hemorrhage and exudation. The main clinical symptoms of the patients are various degree of impaired vision. Clinically, it is often confused with type 1 macular telangiectasia and type 3 macular neovascularization. A thorough understanding of the clinical features of PEVAC is particularly important for its differential diagnosis. Due to the unclear pathogenesis of PEVAC, there is no specific treatment for the cause of disease. Most scholars use intravitreal injection against vascular endothelial growth factor drugs for treatment, but can not improve patients' visual acuity. At present, many attempts have been made to eliminate abnormal exudation of the lesion, maintain visual function and achieve a good prognosis by simple or combined laser photocoagulation. At present, it is still necessary to explore the pathogenesis of PEVAC, improve the understanding of the disease, and find a better treatment plan.