Malignant peripheral nerve sheath tumours are rare soft-tissue tumours consisting of a spindle-cell malignancy arising from a peripheral nerve. Most of these tumours arise on the extremities and are rare in the head and neck region. A 33-year-old woman patient was referred to our hospital with progressive nasal obstruction and discharge occasionally mixed with blood since 1 year. Anterior rhinoscopy revealed a polypoid mass completely occupying the right nasal cavity, with a grossly left-deviating nasal septum. Computed tomography of the paranasal sinuses revealed an expansile soft-tissue mass filling the right nasal cavity, which extended to the right maxillary sinus with an erosion of the right lamina papyracea and an extension to the right orbit. Biopsy revealed the mass to be a malignant peripheral nerve sheath tumour, which was confirmed by immunohistochemical tests. The tumour was removed under general anaesthesia by nasal endoscopy and right medial maxillectomy. The patient recovered uneventfully and was discharged on day-6 after. She was given chemotherapy and is under regular follow-ups, with no recurrence of the disease since 2 years.
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