Computed tomography demonstrated contrast-enhancing lesions in the periventricular frontal regions, caudate nuclei, and thalami in an infant with Alexander's disease. The distribution of the enhancing lesions corresponded to the areas in which Rosenthal fibers were most prominent. These radiological findings have not been described in other white matter diseases; thus, they may help to distinguish Alexander's disease from Canavan's disease and decrease the necessity for diagnostic brain biopsy.