Background: Malignant peripheral nerve sheath tumours (MPNST) are uncommon but aggressive neoplasia associated with radiation exposure and neurofibromatosis (NF). These tumours are often found in the trunk, extremities, head and neck, with capacity to metastasize. Only a handful of case reports have described intramedullary spinal cord MPNST. Methods: We report the case of a 35 year-old female who presented with progressive gait disturbance and paraparesis. MRI of the spine demonstrated an enhancing intramedullary mass at the C7 vertebra. Laminectomy with expansile duraplasty, and extended surgical biopsy were performed for cord decompression and tissue diagnosis. Results: Pathological sections demonstrated a spindle cell neoplasm with nuclear atypia, frequent mitotic figures, focal necrosis, and infiltration into adjacent neurological tissue. It was positive for S100, SOX10, p53 with partial loss of INI-1. Diagnosis of spinal intramedullary MPNST was confirmed, however given the prior history of remote trigeminal MPNST, it was unclear whether the mass represented a delayed metastasis, or a de novo neoplasm. Conclusions: Typically relegated to the periphery, our case represents a rare spinal medullary presentation of MPNST. While the differential for tumours in this location typically includes diffuse astrocytomas, ependymomas, and rarely schwannomas, we move that consideration of MPNST in select high risk cases advise surgical planning and subsequent therapy.