Pachychoroid neovasculopathy (PNV) is a novel clinical entity with typical pachychoroid features accompanied by type 1 choroidal neovascularization (CNV). Typical optical coherence tomography finding of the disease is flat irregular pigment epithelial detachments (double layer sign). Type 1 CNV between retinal pigment epithelium and Bruch membrane is seen as a tangled network on optical coherence tomography angiography. Differential diagnosis of PNV from neovascular age-related macular degeneration is especially important because of the different epidemiological, genetic, and therapeutic features of these diseases. Current treatment approaches include intravitreal anti-vascular endothelial growth factor injections and photodynamic therapy.