Krabbe's disease whose CT appearance is well known should benefit of the development of MRI as this method is more accurate. MRI permits early diagnosis of leucodystrophy. Yet it must be emphasized that abnormal white matter patterns are not sufficient to permit the diagnosis of Krabbe disease. In the case reported atrophy of the brain, the cerebellum and the brain-stem, demyelination of the white matter and necrosis of corpus callosum were observed. The abnormalities of the brain on MRI pictures help in the diagnosis as they may alert clinicians to the possibility of Krabbe disease in infants with progressive encephalopathy. A definitive diagnosis can be established thanks to the laboratory tests.