Ganglioneuroma is a rare benign tumour that originates from the ganglion cells of the sympathetic nervous system. They are rare in cervical spine region and only 8 % of ganglioneuromas occur in the neck. The common sites of occurrence are in the posterior mediastinum, retroperitoneum and adrenal medulla, and as such, a cervical occurrence presenting with quadriplegia is a reportable event. We present a 26-year old young male with a two-year history of neck pain and progressive quadriplegia. He later became wheelchair-bound. Musculoskeletal examination revealed multiple generalized nodular skin swellings with café au lait macules. Magnetic resonance imaging showed a huge dumbbell tumour of the first two cervical vertebrae, to the right side of the spinal canal causing significant spinal cord compression. He had surgical intervention, aimed at complete tumour resection, postoperatively, power of the limbs improved to normal. Histological examination was consistent with ganglioneuroma. We present this report because the occurrence of ganglioneuroma is rare, secondly a cervical presentation is unusual and thirdly it presented as a rare cause of quadriplegia.