Background: When MRI and magnetic resonance spectroscopy (MRS) are used together, they allow for the correlation of anatomical and pathological features in a selected region of the brain. Aim: This study aimed at describing the variable changes in the brain metabolites' levels and ratios in the pediatric white matter diseases. Patients and methods: MR spectroscopy of the affected white matter and basal ganglia was held on 22 eligible infants and children with clinically and radiologically proven white matter diseases. According to the established diagnosis, the cases were classified into five groups including: hypomyelination, inborn errors of metabolism, congenital muscular dystrophies (CMD), and secondary demyelinating disorder.  Results: All the cases of CMD (5 cases) showed decreased Cr level, while only one third of the cases of hypomyelination showed decreased Cr level. Increased mIn level in the affected white matter was seen in 8 cases, half of these cases were finally diagnosed with CMD, however these findings were statistically insignificant. Cho/NAA ratio was increased in the affected white matter in half of the cases diagnosed with CMD and half of the cases diagnosed with hypomyelination. Lactate levels in the basal ganglia showed statistically significant difference among the different groups of disorders, as lactate level was high in the basal ganglia of about two thirds of the cases of inborn errors of metabolism. Conclusion: MRI is the modality of choice in evaluating pediatric white matter disorders, however MR spectroscopy have an adding value in narrowing the differential diagnosis list, define single appropriate diagnosis, or even direct specific investigation.