Cranial computed tomography of a boy with clinical characteristics of the classic form of Pelizaeus-Merzbacher disease did not reveal a notable abnormality other than enlarged ventricles. Magnetic resonance imaging, however, demonstrated diffuse changes in the white matter with sparing of scattered small areas, suggesting persistent myelin islands which are a typical neuropathologic finding in Pelizaeus-Merzbacher disease. Magnetic resonance imaging appears more useful than computed tomography in confirming the diagnosis of Pelizaeus-Merzbacher disease.