Polypoidal choroidal vasculopathy (PCV) is currently recognized as a neovascular exudative maculopathy with features similar to age-related macular degeneration (AMD), including RPE detachment (PED) and the presence of sub and intra-retinal fluid. The pathogenesis of PCV remains unknown. The main current theory suggests that it is a subtype of AMD or a subtype of the pachychoroid spectrum diseases. Unlike AMD, PCV is more common in non-Caucasian populations (including blacks, Hispanics and Asians). The disease usually occurs between the ages of 50 and 70. PCV is usually unilateral but can happen in both eyes. The main risk factors described for PCV are arterial hypertension, chronic kidney disease and smoking. Clinically, PCV is characterized by exudative, serosanguineous PED, RPE/retinal atrophy and disciform scarring. On clinical examination, the polyps are seen as reddish-orange internal projections of the RPE, mainly in the macular region, but may also occur in the peripapillary region or even in the retinal periphery. The ancillary tests are FA, ICG angiography, OCT and OCTA, with ICG being the gold standard for the diagnosis of the disease. Typical ICG angiography observes the presence of neovascular network associated with polypoidal aneurysmal dilatations. The main differential diagnosis is AMD and central serous chorioretinopathy (CSC). Treatment options include thermal laser photocoagulation, photodynamic therapy, antiangiogenic agents, or combination therapy. Antiangiogenic monotherapy is often considered the first-line treatment. Depending on the extent of area involved, prognosis is generally good.