To expand the clinical spectrum of idiopathic polypoidal choroidal vasculopathy based on historical cases and newly recognized observations. A review of the previously reported 45 cases was carried out. An additional 20 cases were retrospectively reviewed to examine the clinical nature and course of idiopathic polypoidal choroidal vasculopathy. New observations on the clinical spectrum of idiopathic polypoidal choroidal vasculopathy were noted for demographic features, the nature and course of the vascular lesion, the possible association with intraocular inflammation, and the indocyanine green angiographic characteristics. Idiopathic polypoidal choroidal vasculopathy seems to be a distinct clinical entity that has a predilection for individuals of pigmented races. The disorder should be differentiated from typical choroidal neovascularization and other known choroidal degenerative, inflammatory, and ischemic disorders because of differences in clinical course and treatment.