Purpose <p>To investigate the neuro-ophthalmologic characteristics, potential triggers, and treatment responses of visual snow syndrome (VSS) in a Korean population.</p> Study design <p>Retrospective case series.</p> Methods <p>We retrospectively reviewed the data of patients diagnosed with VSS at a tertiary hospital from March 2021 to February 2024. Data on visual and nonvisual symptoms, self-reported events that caused VSS, and medical and psychiatric comorbidities were chart-reviewed. Neuroimaging findings from MRI and ¹⁸F-FDG PET were evaluated, along with treatment responses to pharmacological interventions and filter glasses.</p> Results <p>The sample comprised 27 men and 36 women, with a mean age of 27±11 years (mean±SD) and onset age of 22.4±11 years. Common symptoms included floaters, palinopsia, anxiety, and depression. Fourteen participants attributed VSS onset to specific ophthalmic events (e.g. refractive surgery or ophthalmic examinations with bright-light). ¹⁸F-FDG PET scans showed hypermetabolism in the visual cortices (81.8%), with no significant MRI abnormalities. Lamotrigine (18.9%), alprazolam (20%), and filter glasses (32.1%) showed modest efficacy in reducing the intensity of VSS.</p> Conclusion <p>Korean patients with VSS exhibited clinical features, neuroimaging findings, and treatment responses similar to those reported in Western populations. Ophthalmic procedures, such as refractive surgery and bright-light examinations, may act as potential triggers in susceptible individuals. Although functional imaging, including ¹⁸F-FDG PET, may be useful for evaluating VSS, further studies with healthy controls and quantitative analyses are needed to validate its diagnostic value. Given the modest treatment efficacy, a multidisciplinary approach combining pharmacological intervention, filter glasses, and psychological support is essential to optimize outcomes.</p>