Patients with neurofibromatosis 1 (NF1) are predisposed to develop central nervous system tumours, due to the loss of neurofibromin, an inactivator of proto-oncogene RAS. We present a case of NF1 patient with a spinal cord ependymoma. She presents with 3 months history of increasing weakness in right Upper limb and lower limb associated with numbness with involvement of left half of the body since 15 days. Magnetic resonance imaging revealed heterogeneously enhancing solid cystic mass lesion in the intramedullary compartment of the cord extending from C2-C3 to D2-D3 levels with cord edema up to medulla and D11 level craniocaudally respectively, likely to represent ependymoma.