Background: Gangliocytoma is a WHO grade 1 tumour which is typically found in the brain. Incidence of this pathology occurring in the spinal cord is rare, accounting for 0.1%-0.4% of intramedullary spinal cord tumors. Methods: We present the case of a previously healthy 23-year-old male with bilateral weakness and accompanying sensory deficits progressing over several months. Magnetic resonance imaging (MRI) of the spine showed a C3-C6 enhancing, intramedullary mass. C3-6 laminoplasty and resection of the tumour was preformed under neurophysiologic monitoring. Post-operatively the patient had no new neurologic deficits. Results: Final histopathology was consistent for gangliocytoma with extensive calcifications as confirmed by two separate neuropathologists. Gross total resection was not possible due to decreased motor evoked potentials detected by electrophysiologic monitoring. Post-operative MRI revealed a residual tumour measuring 58×15×25 mm with no further abnormal foci of enhancement. Conclusions: Gangliocytoma of the cervical cord is exceedingly rare, however its natural history is unclear. In our experience, maximal safe resection is warranted with the goal of preserving neurological status.