A very rare case of intradural extra-arachnoid schwannoma involving the upper cervical spine is described. The tumour most likely originated from the extra-subarachnoid angle region, with its unusual pattern of expansion within the spinal canal resulting from such characteristics of the upper cervical region as a relatively wide spinal canal, an extensive ventral subarachnoid space, and membranous ventromedian and lateral anchoring structures which influenced the direction of tumour enlargement.