Glioneuronal tumors with neuropil-like islands (rosetted glioneuronal tumors) were recently reported as a novel brain tumor entity with characteristic clinicopathological features (Am J Surg Pathol 23: 502, 1999). Here we describe the clinical, histological and genetic features of another case arising in the parietal lobe of a 43-year-old man suffering from focal motor epilepsy. Histologically, nodules of small neuronal tumor cells immunoreactive for synaptophysin and NeuN were embedded within a diffuse astrocytoma. Remarkably, highest proliferative activity was observed within the neuronal nodules. Comparative genomic hybridization revealed a gain of chromosome 7q and a loss on chromosome 9p.