The MR findings in an adult patient with globoid cell leucodystrophy (GLD) or Krabbe's disease are presented. MRI showed a bilateral periventricular hyperintensity of the parieto-occipital white matter on the T2-weighted images. A hyperintense signal was seen bilaterally along the corticospinal tract. There was no immediate nor delayed contrast enhancement. The MR findings in this case of GLD are rather atypical.