Malignant Peripheral Nerve Sheath Tumour (MPNST) also termed as spindle cell malignancy of the peripheral nerve schwann cell or neurogenic sarcoma represents 10% of all soft tissue sarcomas. The tumour is usually found in lower extremities and only 10% to 20% of all lesions occur in head and neck region thus making it a rare entity. Central involvement, particularly in the jaw bones is quite unusual. Neurofibroma is one of the common nerve sheath tumours occurring in the soft tissues and generally appears in neurofibromatosis I (NF-I or von recklinghausen's disease). MPNST are uncommon sarcomas that almost always arise in the soft tissues. Here we report a case of intraosseous peripheral nerve sheath tumour occurring in the mandible and discuss the surgical management with adjuvant and neoadjuvant treatment plan.

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