Patent foramen ovale (PFO) is a congenital heart anomaly with persistent non-closure of the atrial septum that generally closes six to 12 months after birth in the majority of adults. While remaining asymptomatic in the majority of cases, PFO could lead to paradoxical embolism and cryptogenic strokes in most symptomatic cases. The incidence of small arterial occlusion due to paradoxical emboli is quite uncommon. In this report, we present a case of a 51-year-old man who presented with acute left-sided painless visual loss due to central retinal artery occlusion (CRAO). Stroke work-up and hypercoagulability evaluations were negative. The patient was found to have PFO with the initial presentation as CRAO, a rather rare presentation in the setting of PFO. In this report also, we discuss the clinical presentation, pathogenesis, and the current evidence-based therapeutic options in the management of PFO in adults, highlighting the importance of considering this diagnostic entity in the setting of acute visual loss, as with our case presentation.