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From January 2011 to December 2017, nine consecutive patients with Susac syndrome and a control group of 73 patients with multiple sclerosis or clinically isolated syndrome were included. Two neuroradiologists blinded to the clinical and ophthalmologic data independently reviewed MRIs and assessed leptomeningeal enhancement and parenchymal abnormalities. Follow-up MRIs of patients with Susac syndrome were reviewed and compared with clinical and retinal fluorescein angiographic data evaluated by an independent ophthalmologist. Patients with Susac syndrome were significantly more likely to present with leptomeningeal enhancement: 5/9 (56%) versus 6/73 (8%) in the control group. They had a significantly higher leptomeningeal enhancement burden with ≥3 lesions in 5/9 patients versus 0/73. Regions of leptomeningeal enhancement were significantly more likely to be located in the posterior fossa. The authors conclude that leptomeningeal enhancement occurs frequently in Susac syndrome and could be helpful for diagnosis and prediction of clinical relapse.