The review addresses MR imaging of hereditary leukoencephalopathies (HL) as one of the most urgent problems of modern neurology and neuroradiology. HL is a group of rare human diseases that occur with a primary brain white matter lesions. Modern theoretical and methodological approaches for MRI of patients with hereditary leukoencephalopathies and data analysis are discussed. The authors demonstrate that MRI plays the important role in the diagnosis and differential diagnosis of HL, in-vivo imaging of structural changes in the brain that are correlated with pathogenetic mechanisms of these diseases.