We present the case of a 13-year-old boy who had a 12 year history of complex partial seizures. Recent CT scan and Magnetic Resonance Imaging (MRI) showed a contrast-enhancing lesion of the left temporal lobe, while CT performed 6 years ago was unconspicuous. Extended lesionectomy was performed according to the results of presurgical epileptological evaluation. Histological examination of the specimen showed an anaplastic ganglioglioma (WHO grade III). The clinical, neuroradiological, and histopathological findings of our patient are presented with respect to the literature on malignant gangliogliomas.