Abstract
Dysembryoplastic neuroepithelial tumour (DNET) is an uncommon mixed glioneuronal tumour. DNET is classified as Grade I neoplasm in revised World Health Organization classification of tumors of the nervous system. DNET is commonly seen in the temporal lobe of children and young adults with features of pharmacoresistant complex partial seizures. Tumors arising in association with DNETs are rare. Only two cases of pilocytic astrocytoma (PA) arising in DNETs are reported. Surgical excision is the only successful management with favourable prognosis. The development of recurrence and malignancy after subtotal or even after complete excision challenges the premise of stability and highlights the importance of close clinical follow up. Here, a case of DNET with area of PA is described which helps in understanding the pathogenesis and biological behavior of DNET.