Fourteen children with Epstein-Barr virus (EBV) encephalitis admitted to our pediatric department during the period 1988 to 1998 were collected and reviewed to characterize the clinical, laboratory and neuroradiological findings. There were 7 boys and 7 girls. The age of onset ranged from 10 months to 14 years. Among them, 5 patients belonged to Alice in Wonderland syndrome, 5 were diagnosed as acute viral encephalitis, 1 presented with acute meningoencephalitis followed by cerebellitis, the remaining 3 cases attributed to acute disseminated encephalomyelitis. The main symptoms were fever (43%), seizure (36%), bizarre behavior (31%), headache (21%) and metamorphopsia (36%). The presenting signs included altered consciousness (50%), meningeal sign (14%), bulbar sign (14%), cerebellar sign (7%), and cranial nerve palsy (7%). Classic findings of infectious mononucleosis were obscure. The laboratory data showed the existence of atypical lymphocyte in only one case but positive serology for EBV infection in all patients. Pleocytosis was found in 3 (30%) of 10 patients examined. Eight (67%) of 12 patients had nonspecific electroencephalographic changes in the acute stage. Computed tomography (CT) scans were abnormal in 2 (40%) of 5 patients tested; while magnetic resonance image (MRI) disclosed lesions in 5 (56%) of 9 patients, with abnormal signals in various parts of the brain. Single photon emission computed tomography (SPECT) brain scan showed abnormal perfusion lesions in 3 (75%) of 4 patients studied. The results demonstrate the diversity of neurological manifestations of EBV encephalitis. EBV should be considered in any acute neurological illness of uncertain etiology in the pediatric population. While MRI remains the image of choice in EBV encephalitis, SPECT detects the abnormal perfusion more precisely in a substantial number of patients.