Idiopathic parafoveal telangiectasis (IPT) is a kind of disease which is rare, with potential clinical blindness, sometimes confused with some other macular diseases, and its pathogenesis is not yet clear. According to the different classification, main clinical manifestations are macular telangiectasis, leakage, or neovascularization, and visual loss resulted from gradual retinal atrophy. The clinical characteristic in different stages of this disease, progress of multimodal fundus imaging technology in diagnosis and recent development of treatment methods were reviewed in this paper.