RESUMO
Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue sarcomas, with 50% of cases associated with type 1 neurofibromatosis (NF-1). A 27-year-old male patient was referred to our department with an extended right nasal cavity MPNST. The lesion extended to the skull base, intracranial, parapharyngeal space, and infratemporal fossa. NF-1 was also confirmed by a neurologist. The patient was negative for distant metastases. Due to the tumor's proximity to vital structures, it was decided to treat it with chemotherapy and radiotherapy. Nasal cavity MPNSTs are particularly uncommon, with few reported cases. They should be included in the differential diagnosis of nasal masses or recurrent nosebleeds, particularly in patients with NF-1. Careful follow-up is essential to detect early recurrence, which contributes to a better prognosis.