'Perifoveal Exudative Vascular Anomalous Complex' (PEVAC) is a perifoveal, unilateral, isolated, perifoveal aneurysm, in otherwise healthy patients. Here, we report a case of PEVAC in a highly myopic eye of a 86-year-old woman affected by a visual decline in the right eye (best-corrected visual acuity of 20/100). She had no other relevant past conditions and/or ocular impairment. Fundus examination in the right eye showed myopic chorioretinal degeneration with a concomitant PEVAC. Structural optical coherence tomography (OCT) showed a round lesion with a hyperreflective wall associated with intraretinal cystic spaces. OCT-angiography nicely disclosed an isolated large aneurysmal retinal dilation featuring the PEVAC with detectable flow in superficial capillary plexus, deep capillary plexus, and avascular slab. This case highlights the importance of discerning between different vascular disorders of the macula, in order to be able to offer the right treatment and/or follow-up to the patient.