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Background Primary spinal epidural lymphoma (PSEL) is one of the rarest categories of tumors. Spinal cord compression is an uncommon primary manifestation and requires to be treated with surgery for the purpose of diagnosis and decompression. Case Presentation A 45-year-old man presented with a new onset thoracic pain and progress to an anterior spinal syndrome with hypoesthesia and loss of thermalgesia. Magnetic resonance image showed a paravertebral mass that produces medullary compression at T3. The patient was taken up to surgery, where the pathology examination showed a diffuse large B-cell lymphoma. Conclusions PSEL is a pathological entity, which must be considered on a middle-aged man who began with radicular compression, and the treatment of choice is decompression and biopsy. The specific management has not been established yet, but the literature suggests chemotherapy and radiotherapy; however, the outcome is unclear.

HighlightsIntracranial malignant peripheral nerve sheath tumors are an extremely rare pathology with a high morbidity and mortality.The most frequently affected cranial nerves in decreasing order of frequency are the trigeminal, vestibulocochlear and facial, being glossopharyngeal, vagus and spinal nerves extremely rare affected.This manuscript is the first in English-written literature to expose the case of an adult female patient with NF1 who presented a MPNST involving the glossopharyngeal, vagus and spinal left nerves with intracranial-extracranial extension and systemic metastases.Treatment strategies for this particular pathology are still unclear due to the low frequency of the disease and a lack of number of cases, making them a challenge for the neurosurgeon.

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