Introduction: Canavan disease is a rare autosomal recessive leukodystrophy caused by pathogenic variants in the ASPA gene, resulting in aspartoacylase deficiency and progressive spongiform degeneration of cerebral white matter. It typically manifests in early infancy with hypotonia, macrocephaly , seizures, and severe developmental delay . However, clinicalvariability and overlap with acquired neurological insults may obscure early diagnosis. Recognition of characteristic neuroimaging patterns is critical, particularly in nonconsanguineous families. Case presentation: A 1-year-9-month-old male born at term to non-consanguineous parents was evaluated for global developmental delay and regression following bronchopneumonia with presumed meningitis at 4 months of age. Development had been normal prior to this illness. At presentation, he was unable to sit even with support but could roll laterally . Partial recovery of previously lost head control and palmar grasp was noted. Social interaction and babbling were preserved, and no definite seizures were documented. Examination revealed generalized hypertonia, exaggerated reflexes, dystonia, and nystagmus. Occipitofrontal circumference measured 49 cm (+1 SD). Brain MRI demonstrated markedly delayed myelination, limited to the posterior limb of the internal capsule, with T2 hyperintensities involving the posterior corpus callosum and bilateral globus pallidus. Genetic testing confirmed a pathogenic ASPA mutation. Conclusions: This case highlights the importance of considering leukodystrophy in infants presenting with apparent postinfectious regression. Even in the absence of seizures or significant macrocephaly , characteristic MRI findings should prompt genetic evaluation. Early molecular confirmation enables accurate prognostication, anticipatory multidisciplinary management, and essential genetic counseling for recurrence risk.