PURPOSE: The purpose of this study was to analyze the MR findings of childhood adrenoleukodystrophy regarding the extent of white matter degeneration and the pattern of enhancement, and to correlate these findings with clinical manifestations. MATERIALS AND METHODS: Six male patients (5-13 years old;mean age, 8.7 years) of biochemically confirmed adrenoleukodystrophy were included in this study. We evaluated the extent of white matter degeneration which was shown as high signal intensity on T2-weighted image, and the pattern of enhancement. Also we correlated the MR findings with clinical manifestations of symptoms and the results of evoked potential studies. RESULTS:The most common sites of predominant involvement were occipital deep white matter, optic radiation, and splenium of corpus callosum (n = 4, 66.7%). The other sites of predominant involvement were frontal deep white matter (n = 1, 16.7%) and pyramidal tract with cerebellum (n = 1, 16.7%). After contrast enhancement, there was peripheral rim enhancement surrounding the most severely affected areas in five cases (83. 3%). These findings were consistent with clinical symptoms in five cases (83.3%), but the results of evoked potential studies were consistent with clinical symptoms in only two cases (33.3%). CONCLUSION:In childhood adrenoleukodystrophy, MRI can clearly demonstrate specific tracts of involvement and these findings were well correlated with clinical manifestations.