Objective Malignant peripheral nerve sheath tumors are uncommon malignant spindle cell tumors that account for 5% to 10% of all soft tissue sarcomas. Localization of such tumors in the scalp is extremely rare. Diagnosing and treating these tumors is very challenging. Methods Case presentation and Literature review. Results We found 18 cases of malignant peripheral nerve sheath tumors including our case. The occipital region was the most common site of the tumor. Five patients had h intracranial

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... invasion and two patients had distant metastasis. All the patients were treated surgically. Four patients had recurrence of their tumors and two of them died from the disease Conclusion MPNSTs should be considered in the differential diagnosis of rapidly enlarging scalp tumors. Radical excision with wide margins and adjuvant radiation therapy should be considered as the standard treatment for these highly malignant tumors