Abstract: Objective To observe the characteristics and treatment of malignant peripheral nerve sheath tumor originated from neurofibromatosis and the outcome of the patients. Methods A case was reported and the related literatures were reviewed. Results and Conclusion Malignant peripheral nerve sheath tumor is a rare soft tissue malignancy, which is with highly invasion. There are some difficulties with the diagnosis and treatment, and the outcome is poor. Early diagnosis is very important for treatment.

Key words: malignant peripheral nerve sheath tumor, neurofibromatosis, treatment, outcome