Ganglioneuroblastoma in the spinal region is rare, the treatment of choice being surgical excision. We present a 21 year old male who was diagnosed to have this condition in the dorsolumbar spinal region. The tumour was extending intraspinally and was unresectable. Combination chemotherapy with Adriamycin® (doxorubicin hydrochloride), vincristine, cyclophosphamide, etoposide, ifosfamide and cisplatin resulted in histologically proven complete remission. No radiotherapy or curative resection was done. The patient is alive without evidence of disease 24 months later. Never before has chemotherapy been successfully used as the sole modality of treatment in this condition. Our report raises important questions about the management of this rare condition, particularly in a situation of unresectability.