**Abstract**

Neuroblastoma is one of the most common solid tumour in the paediatric age group. Central nervous system (CNS) involvement in neuroblastoma is commonly due to metastasis from the extracranial primary. Primary CNS Neuroblastoma (PCNS-NB) is a rare entity and highlights errors in development of neural crest cells and CNS. A lot has been published since the first description of PCNS-NB four decades ago. Over the years, neuroscientists, geneticists, and clinicians have improved the understanding of PCNS-NB. PCNS-NB is an enigmatic entity with variable presentation, epidemiology, clinical features and outcomes. Recent update in knowledge is seen in 2016 WHO classification of CNS tumours with reclassification of CNS neuroblastoma. It further subclassified different histological variants of PCNS-NB and its molecular correlates. Most common histological subtype of PCNS-NB is neuroblastoma followed by ganglioneuroblastoma. Studies support the view that younger age group, less number of lesions, ganglioneuroblastoma histology subtype and surgical management are good prognostic indicators. This chapter provides an updated overview of epidemiology, clinical features, histological and molecular diagnosis, and outcomes of PCNS-NB in addition to the role of adjuvant therapy.

**Keywords:** Primary central nervous system neuroblastoma, Ganglioneuroblastoma, CNS PNET, CNS Neuroblastoma with FOX-R2, CNS embryonal tumours
