We report a case of 46-year-old xanthoderm woman who was diagnosed as malignant peripheral nerve sheath tumors of right maxillary sinus, and have a literature review. Histology confirmed a diagnosis of malignant peripheral nerve sheath tumor. The woman had the right total maxillectomy and postoperative adjuvant radiotherapy. There is no local recurrence or metastasis of one year following up. Literature review revealed MPNST in the nasal cavity and para-nasal sinuses were not common with poor prognosis. The main cause of death is local recurrence and metastasis. Surgical resection showed more advantage than adjuvant radiotherapy and chemotherapy.