We report a 46-year-old patient with Lhermitte-Duclos disease (LDD) who underwent a successful surgery but died of other causes four years later. The autopsy revealed Lhermitte-Duclos disease asymmetrically affecting both cerebellar hemispheres. The subcortical white matter of both cerebral hemispheres contained several foci of grey matter heterotopia. Only heterotopic neurons contained tau-positive neurofibrillary tangles (NFT), displaying characteristic ultrastructural features of paired

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... al filaments (PHF). Neither senile plaques nor NFTs were found in other areas of the central nervous system. The brain also showed other developmental abnormalities such as megalencephaly, numerous foci of meningeal glial heterotopia and multifocal telangiectasia. Although some of these findings were previously described in LDD, this is the first case of this disease with NFTs selectively accumulating in the neuronal heterotopic tissue.