Retinal angiomatous proliferation (RAP) is a genetic distinct subgroup of exudative age-related macular degeneration which shows a rapid and severe vision loss and high recurrence rates. The pathophysiological mechanisms of RAP is unclear. Recent histopathologic study and en face optical coherence tomography angiography have furthered our understanding of RAP. Clinical features frequently associated with RAP include bilateral disease, presence of reticular pseudodrusen and pigment epithelial detachments. Indocyanine green angiography is the gold standard diagnostic tool. Recently, more and more accurate optical coherence tomography has improved the acknowledgement of stage and diagnosis of RAP. The treatment efficacy of RAP is highly dependent on the stage. Anti-vascular endothelial growth factor therapy is currently the first line of treatment. Other treatment options including combination of photodynamic therapy with antiangiogenic agent intravitreal injections also achieve a reasonable therapeutic outcome. There remain several important questions such as pathogenesis and treatment regimen, to be answered in future RAP research studies.