Background: Acute disseminated encephalomyelitis (ADEM), which occurs spontaneously or after systemic viral infection and vaccination mostly affects children. This study aims at describing the epidemiologic, clinical, neuroimaging, laboratory features, treatment and outcome in children who diagnosed as having ADEM and admitted in a referral center in Tehran. Methods: This descriptive prospective study was conducted on patients with a diagnosis of ADEM over a period of 30 months, between Sep 2003 and Mar 2006, admitted to the neurology ward of Mofid Pediatric Hospital. All these patients were visited in regular follow-up every 6 months for two years. Results: Eighteen patients with the mean age of 6 years (range 5 months - 12 years) were studied. No sex predominance was noted. Sixty-one percent had prodromal illness, and two patients had been vaccinated before the illness. Thirteen (72%) patients presented in fall or winter. Motor deficits and cranial nerve palsy were the most common features, which had occurred in 13 (72%) patients. Abnormal findings in cerebrospinal fluid evaluation were detected in 33% of the patients. Brain computed tomography was normal in all but one patient. Electroencephalograms done in nine patients were normal in 54%. Magnetic resonance imaging showed lesions were most commonly in the subcortical and periventricular areas (76%). Nine (50%) patients were treated with corticosteroids and intravenous immunoglobulin. The mortality rate was 5.5%, and the relapse occurred only in one case. In two-thirds of the patients, prognosis for complete recovery was excellent. Conclusion: Childhood ADEM is a benign condition, affecting both sexes equally. Recurrent infections have been its common cause in our center.