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Neuroblastic tumours are common in childhood and adrenal glands are the most common site. Head and neck ganglioneuroblastomas are extremely rare and nose is a very uncommon site for a ganglioneuroblastoma. The management of this primitive sympathogonic tumour may vary depending on the age of the patient and stage of the tumour. We present a middle-aged man with a ganglioneuroblastoma of skull base, management of this tumour and a review of literature.