PURPOSE: Acute exudative polymorphous vitelliform maculopathy (AEPVM) is a rare retinal disorder first described by Gass in 1988. Characterized by multifocal yellow-white lesions at the level of the retinal pigment epithelium (RPE) and associated with serous retinal detachments, AEPVM typically presents with acute visual disturbances and, in some cases, headaches. Despite its rarity, with fewer than 40 cases reported in the literature, its clinical course and etiology remain poorly understood. Patients generally experience gradual recovery of vision, though retinal abnormalities may persist. Various hypotheses suggest infectious, inflammatory, autoimmune, or even paraneoplastic mechanisms, but definitive causes remain elusive. In this report, we describe the clinical course and multimodal imaging findings of a patient diagnosed with AEPVM, contributing to the limited understanding of this condition's progression and management. CASE PRESENTATION: We report the case of a 40-year-old Caucasian female presenting with blurred vision for two weeks, with a best corrected visual acuity of 20/20 in both eyes, preceded by flu-like symptoms. Initial clinical evaluation, including fundus examination and spectral-domain optical coherence tomography (SD-OCT), revealed multiple small serous retinal detachments bilaterally. Multimodal imaging (fundus autofluorescence, fluorescein angiography, and indocyanine green angiography) appeared normal initially. A comprehensive systemic workup excluded autoimmune, infectious, and neoplastic etiologies. Upon follow-up, yellowish retinal lesions and hyperautofluorescence emerged, leading to the diagnosis of idiopathic AEPVM. The patient continues to be monitored for visual recovery and potential complications. CONCLUSION: AEPVM is a rare macular disorder that requires comprehensive multimodal evaluation to establish an accurate diagnosis. Ruling out autoimmune, infectious, and especially neoplastic causes, including paraneoplastic syndromes, is critical for confirming its idiopathic nature. While visual recovery is common, the risk of recurrence and complications such as choroidal neovascularization necessitates vigilant long-term monitoring.