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We describe a case of extrapontine myelinolysis in a child presenting with chorea and parkinsonian symptoms after treatment for hyponatremic dehydration. Although both extrapontine myelinolysis (EPM) in childhood and extrapyramidal presentation of EPM are very rare in children, the patient presented with pure extrapyramidal symptoms. Besides basal ganglia involvement and sparing brain stem, MRI demonstrated involvement of the amygdala bilaterally hitherto never reported in EPM patients. While single voxel proton spectroscopy (MRS) of the basal ganglia at the beginning was normal, the follow-up MRS showed moderate to severe NAA and mI decrease. Diffusion-weighted imaging abnormalities appeared relatively late during the disease course, also an unexpected finding. In addition, there were apparent increases in apparent diffusion coefficient (ADC) values of the affected basal ganglia instead of diffusion restriction.