Magnetic resonance (MR) spectroscopy is an analytical method used as an adjunct to the more common method of magnetic resonance imaging (MRI) to study metabolic changes in various brain diseases, such as hereditary leukodystrophies. Object and methods of the study. Analysis of MR spectroscopy protocols of 3 patients with MR signs of leukodystrophy. Results. As a result of comparing MR spectroscopy and MRI data of a 20-year-old patient with epileptic seizures, progressive cognitive deficit and unsteadiness of gait, an increase in the lipid peak in pathological areas is noted, which indicates a possible mitochondrial encephalopathy. Genetic study revealed a mutation in the MT-ND5 gene. In a 23-year-old patient with moderate spastic lower paraparesis, the MRI pattern meets the Steenweg criteria for the diagnosis of leukoencephalopathy with brainstem and spinal cord damage and increased lactate (LBSL). MR spectroscopy did not reveal an increase in the peak lactate in the medulla (which is a criterion for confirming the diagnosis of LBSL), but the results indicated pronounced processes of active demyelination. Genetic testing revealed 2 pathogenic variants of the DARS2 gene. According to the MR spectroscopy data of a 45-year-old patient with lower limb weakness, dysarthria, cognitive deficits and diffuse damage to the white matter of both cerebral hemispheres, a moderate decrease in N-acetylaspartate and a moderate increase in choline levels in the affected areas, together with MRI data more characteristic of autosomal dominant adult leukodystrophy. Conclusion. MR spectroscopy is of great importance in establishing and verifying the diagnosis in patients with hereditary leukodystrophies.