Focal capillary ectasia in the macular region can manifest in distinct clinical scenarios, which can be categorized into 2 main entities: perifoveal vascular anomalous complex (PVAC) and telangiectatic capillaries (TelCaps). PVAC represents a primary, idiopathic condition, whereas TelCaps occur secondary to underlying vascular disorders, including diabetic macular edema and retinal vein occlusion. We provide a comprehensive analysis of these 2 entities, encompassing their clinical presentations, multimodal imaging findings, histological evidence, and differential diagnosis from other retinal microvascular abnormalities, such as Type 1 macular telangiectasia, adult-onset Coats disease, Type 3 macular neovascularization in age-related macular degeneration, and retinal arterial macroaneurysms. Although PVAC and TelCaps are distinct entities, they may share common pathogenic mechanisms, including progressive endothelial dysfunction, pericyte loss, and intraluminal deposition of blood components. Selective laser photocoagulation has emerged as a promising therapeutic approach for both conditions. The proposed standardization of nomenclature for accurate reporting and meaningful cross-study comparisons is expected to facilitate future advancements in this field, ultimately leading to improved patient outcomes.