Schilder's diffuse myelinoclastic sclerosis, a rarely described disease in childhood, was diagnosed in the case of a 6-year-old girl with subacute cerebral disease. CT-scanning showed asymmetric rim-enhancing lesions within the cerebral white matter. Spontaneous clinical improvement occurred prior to corticosteroid treatment. Follow-up by CT and MRI for three years showed the development of stable amyelinic lesions. Improvement in motor control and in electrophysiological recordings contrasted with an arrest in neuropsychological development. The differential diagnosis and the prognostic implications of this disease are stressed.