Abstract: Polypoidal choroidal vasculopathy (PCV) is a distinct clinical entity characterized by orangered polypoidal subretinal lesions with recurrent serous and hemorrhagic detachments of the retinal pigment epithelium (RPE) and neurosensory retina. Fundus fluorescein angiography (FFA) has no typical manifestations while indocyanine green angiography (ICGA) can show the presence of choroidal networks of branching vessels as well as polypoidal or aneurismal dilations at the termination of choroidal vessels. Optical coherence tomography (OCT) findings often present as sharp protrusions of the RPE and the high reflective layer of choroidal capillaries with inner moderate reflections or nodular lesions. PCV should be differentiated from wet agerelated macular degeneration and some chronic central serous choroidal diseases. Although there is no standard protocol for the treatment of PCV, photodynamic therapy combined with intravitreal antiVEGF therapy can improve or stabilize patient's vision.   (Int Rev Ophthalmol, 2013, 37: 36-42)