Dysembryoplastic neuroepithelial tumour (DNT), a non-evolutive intracr
anial cortical lesion, is generally associated with epileptic seizures
principally among youths. A case of a DNT which presented with uncomm
on clinical features, characterized by severe intracranial hypertensio
n and progressive blindness warranting emergency surgery, is documente
d. In addition to the classical radiological and neuropathological fea
tures characteristic of DNTs there was a large haemorrhagic cystic hae
matoma as a result of repeated dissections and/or ruptures of the abno
rmal vessels in areas, explaining some of the atypical clinical sympto
ms. Therefore the need for a regular, careful clinical and radiologica
l follow-up of cases with cystic DNTs is strongly recommended.