We described the case of an adult who presented with acute cerebellar ataxia (ACA) after Epstein‐Barr virus (EBV) infection. We also reviewed the literature on the pathogenesis of ACA due to EBV infection using the Pubmed database. A 25‐year‐old man suffered from fever, sore throat, and poor appetite. Ataxia with nausea and vomiting developed in the following disease course. Laboratory tests showed atypical leukocytosis and hepatitis. The serological test confirmed a recent EBV infection. Analysis of cerebrospinal fluid (CSF) was unremarkable, so was brain magnetic resonance imaging (MRI). He was diagnosed with infectious mononucleosis and ACA. He fully recovered after conservative treatment. Possible pathogenetic mechanisms of EBV‐associated central nervous system (CNS) manifestations could be categorized as a direct viral invasion of tissue and a postinfectious immune response. Most patients with ACA after EBV infection could be managed with supportive care. The diagnosis of ACA after EBV infection is typically made based on clinical symptoms and signs along with serological detection of EBV‐specific antibodies.