Peripapillary pachychoroid syndrome(PPS)is a novel entity of pachychoroid disease spectrum(PDS)reported online in 2017. PPS eyes displayed thicker nasal versus temporal macular choroids, unlike PDS eyes with thicker temporal macular choroids. Peripapillary intraretinal and/or subretinal fluid was often overlying dilated Haller layer vessels(pachyvessels). Most PPS eyes exhibited other PDS findings including retinal pigment epithelial changes, serous pigment epithelial detachment and outer retinal atrophy. Their optic disc is usually crowded, and some eyes have disc edema and mild late fluorescence leakage. Most of the patients are elderly, and short axial length and hyperopia are common. While some patients experienced visual decline, the overall visual outcome was relatively favourable. Although anti-VEGF or photodynamic therapy were applied, the optimal management remains to be determined. Recognition of PPS is important to distinguish it from disorders with overlapping features such as posterior uveitis and neuro-ophthalmologic conditions. It has been suggested that PDS is consequences of chronic vortex vein stasis. And the hypothesis of choroidal venous overload provides with a unifying concept for pathophysiology of the diseases.