Purpose: The aim of this review was to summarize the most recent investigations on perifoveal Exudative Vascular Anomalous Complex (PEVAC) lesions and guide future research of this entity. Methods: Retrospective analysis of Cochrane Central, PubMed, Web of Science, and ClinicalTrials.gov databases for papers regarding PEVAC, looking for the following keywords: “perifoveal exudative anomalous vascular complex”, “PEVAC”, “PVAC”, “nePVAC”, “ePVAC”, “deep retinal age-related microvascular anomalies” and “DRAMA”. Results: PEVACs are describe as single, massive, unilateral aneurysm, frequently presents with intraretinal cystic edema surrounding the fovea, active exudation and haemorrhages, usually in healthy patients. Notwithstanding this, association with age-related macular degeneration (AMD), myopia, diabetes, lamellar hole and pachychoroid pigment epitheliopathy (PPE) are frequently reported. Currently a multimodal imaging is warrant, especially the use of OCT and OCTA can rule out several other differential diagnoses. Due to the uncertainty of the pathophysiological mechanism the right management of these lesions are yet to be clear. Overall, laser therapy has been effective in several reports, both with thermal and micropulse type. Anti-VEGF injections and steroid injections demonstrated a non-constant response. Conclusion: Several new findings are revolutionizing PEVAC management. Greater standardization could help understand the correct treatment of these lesions.