A 61-year-old man with no history of HIV infection developed a subacutely progressive dementia and left hemiparesis. Brain MRI showed a high intensity lesion in the right frontal lobe on T2 weighted image. There was no contrast enhancement after gadolinium-DTPA administration. 1H MRS revealed a marked decrease in the n-acetyl aspartate/creatine ratios and an increase in the choline/creatine ratio. A lactate peak also was present. A low-grade glioma was suspected and he was admitted to our hospital. On examination, there was a mild dementia and left hemiparesis. A peripheral blood count revealed lymphocytopenia (426/mm3) with a CD4/CD8 ratio of 0.28. No evidence of HIV infection, malignancies or collagen disease was found. A brain biopsy revealed no tumor cells but instead demyelinated brain tissue with large nucleated cells. JC virus antigen was detected in the cells of the demyelinated lesions. A diagnosis of PML associated with idiopathic CD4 positive lymphocytopenia was made. There are only a few reports concerning 1H-MRS findings in patients with PML and the present case illustrates the difficulty of making a differential diagnosis between PML and glioma.