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BackgroundMalignant peripheral nerve sheath tumors (MPNSTs), sarcomas originating from tissues of mesenchymal origin, are rare in patients without a history of neurofibromatosis.Case ReportWe report a case of an MPNST of the spinal accessory nerve, unassociated with neurofibromatosis, which metastasized to the brain. The tumor, originating in the intrasternomastoid segment of the spinal accessory nerve, was removed. Two years later, the patient presented with focal neurological deficits. Radiographic findings revealed a well-defined 2.2×2.2×2.2 cm, homogeneously enhancing mass in the left parieto-occipital region of the brain surrounded by significant vasogenic edema and mass effect, culminating in a 1-cm midline shift to the right. The mass was surgically removed. The patient had nearly complete recovery of vision, speech, and memory.ConclusionsTo our knowledge, this is the first documented case of an MPNST arising from an extracranial segment of the spinal accessory nerve and metastasizing to the brain.