Familial Mediterranean fever (FMF) is characterized by recurrent episodes of fever accompanied by serosal, synovial, or cutaneous inflammation. The central nervous system (CNS) is rarely involved in FMF. The CNS involvement includes demyelinating lesions, posterior reversible encephalopathy syndrome, pseudotumor cerebri, optic neuritis, and cerebral vasculitis. Here, we present a 20-year-old man, a known case of FMF with abrupt left-sided hemiparesis. Brain magnetic resonance imaging revealed right periventricular infarction. Normal echocardiography ruled out cardioembolism, and thrombophilia workup was negative. Therefore, FMF-induced cerebrovascular accident was considered. Although rare, CNS involvement as a result of FMF disease should also be considered when encountering patients with FMF and CNS manifestations.