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Pleomorphic xanthoastrocytoma (PXA) is a rare slow-growing neoplasm that has predilection for the supratentorial compartment and the temporal lobe. Children and young adults are most frequently affected and they usually present with medically refractory seizures. PXAs involving the spinal cord have been rarely documented. We describe a 15-year-old boy who presented with shoulder/neck pain and upper extremity numbness/weakness. Neuroimaging revealed a solid, contrast enhancing, and intramedullary C5 - C6 mass. Microscopy demonstrated a typical WHO grade II PXA. Molecular testing did not reveal a BRAF V600E, IDH1 R132H, or IDH2 R172H mutation. Two years after a near total resection, significant tumor progression was noted via neuroimaging. To the authors' knowledge, this is the first description of the molecular characteristics of a spinal cord PXA.