Epstein-Barr virus (EBV) usually causes mild, asymptomatic, and self-recovered infections in young children. Yet, neurological involvement of this virus has been reported. EBV meningoencephalitis is relatively rare in immunocompetent children. Herein, we describe a case of 2-year-old previously healthy girl presented with high-grade fever and exudative tonsillitis. Her neurological examination showed alteration of consciousness and neck stiffness. A history of generalized tonic-clonic seizures was noted. A diagnosis of EBV meningoencephalitis was definitely confirmed by a positive result for serum viral capsid antigen IgM, and a detection of EBV DNA in cerebrospinal fluid. Her neuroimaging studies demonstrated evidence of leptomeningeal enhancements along bilateral parietal cortical sulci and around the brainstem with a hypodense lesion in the left parietal area - the typical findings of EBV meningoencephalitis. This patient was treated with intravenous corticosteroid without antiviral agents. Her clinical symptoms gradually improved. She was discharged from the hospital on the 19th day of hospitalization without neurological sequelae. Although EBV is not a primary causative agent of meningoencephalitis in immunocompetent children, it should always be considered regardless of the presence or absence of classical infectious mononucleosis symptoms. Early recognition and properly treatment are important for a good prognosis.