This report describes a case with paracentral acute middle maculopathy revealing a giant aortic aneurysm. A 41-year-old man presented with unilaterally unexplained sudden vision loss in the left eye. Fundoscopy revealed a slightly pale r -26etinal reflex in the superior retinal hemifield. SDOCT revealed blurred boundaries between retinal segments and prominent edema in the middle retinal layers. OCTA revealed decreased vascular density in the deep capillary plexus. FA showed low arterial circulation mainly in the superior retinal hemifield. Humphrey VFT revealed an inferior altitudinal defect in the left eye, implying a related circulatory disturbance in the corresponding region.A thorough systemic work-up, including a cardiovascular etiological investigation, revealed giant aortic aneurysm in the ascending aorta, necessitating emergency surgery. This report highlights the importance of a systemic etiological investigation in patients with PAMM to rule out any potential cardiovascular issues.