Idiopathic parafoveal telangiectasis (IPT) is a retinal vascular disease which is characterized by foveal and parafoveal telangiectasia. The main clinical manifestations are retinal telangiectasis, reduced retinal transparency, retinal venular dilatation, yellow exudation, retinal pigment epithelial lesions, retinal hemorrhage, macular atrophy, macular hole or lamellar hole, subretinal neovascularization and retinal detachment. According to the clinical characteristics and features of fluorescein angiography, IPT can be divided into 3 types and 6 subtypes. Laser photocoagulation, photodynamic therapy, and intravitreal injection of glucocorticoid or anti-vascular endothelial growth factor drugs, can reduce the macular edema and neovascularization. However, due to the unclear etiology of IPT, the existing treatment measures are not specific for its etiology. We need to work hard to understand further the clinical features and pathogenesis of IPT and search the targeted treatments based on its pathogenesis mechanism.