Peripapillary choroidal neovascularization is an important feature in the pathogenesis and treatment of a number of disorders affecting the optic nervehead and adjacent chorioretinal areas. Although less well understood than its macular counterpart, peripapillary subretinal neovascularization possesses characteristic clinical and histopathologic features that directly affect its prognosis and management. This review provides a summary of the diseases in which peripapillary subretinal neovascularization occurs and an overview of studies on the natural history, clinicopathologic findings, and therapy. In addition, a pathogenetic model of peripapillary subretinal neovascularization is discussed based on these findings.