Dysembryoplastic neuroepithelial rumours (DNET) are mainly benign cortical
lesions. DNET in the caudate nucleus, thalamus, hypothalamus, pens and cere
bellar hemispheres has also been reported. We describe a fronto-temporo-par
ietal DNET extending to the ipsilateral thalamus and internal capsule, asso
ciated with cerebellar lobe atrophy. Involvement of the internal capsule an
d complication of DNET with cerebellar atrophy have not been reported previ
ously. We emphasise the importance of early diagnosis and treatment of this
rare condition.