The clinical and radiological features of childhood acute transverse myelitis are compared to those of acute disseminated encephalomyelitis with spinal cord involvement in 22 children with acute transverse myelitis and 12 children with acute disseminated encephalomyelitis with spinal cord involvement. Children with acute transverse myelitis were more likely to have a sensory level (55%) and areflexia. Sixty-eight percent of the children with acute transverse myelitis, and 92% of children with acute disseminated encephalomyelitis had longitudinally extensive transverse myelitis. Demyelination was more extensive in acute disseminated encephalomyelitis (mean 15.6 vertebral segments) than in acute transverse myelitis (mean 8.0 vertebral segments). The outcome was normal to good in 82% with acute transverse myelitis and in 100% with acute disseminated encephalomyelitis. Persistent bladder dysfunction was uncommon in both. Poor prognostic factors in acute transverse myelitis are flaccid paraparesis, respiratory failure, and age less than 6 months. These clinical and radiological differences suggest acute transverse myelitis and acute disseminated encephalomyelitis are separate entities.