The efficacy of Haematopoietic Stem Cell-Gene Therapy for Metachromatic Leukodystrophy is under evaluation. CNS damage is currently rated by means of visual MR severity scores. The aim of this study was to investigate whether quantitative spectroscopy measurements correlate with MR severity scores. This preliminary analysis shows that both NAA/Cr and NAA/Cho ratios are associated with progression in total MR Severity Score, Atrophy and Demyelination Load. This suggests that MR spectroscopy could be useful to longitudinally and quantitatively evaluate the disease burden in MLD patients.