Malignant peripheral nerve sheath tumors of the nose and paranasal sinuses are extremely uncommon. We report the case of a 65-year-old woman who presented with a rapid emergence of "nasal polyps" that completely obstructed her nasal passages. Six years earlier, she had been assessed elsewhere for nasal polyps. At presentation, the patient exhibited gross polyposis, with lesions protruding from both nostrils. Histology confirmed a diagnosis of a malignant peripheral nerve sheath tumor. This case demonstrates that symptoms of nasal obstruction are not always secondary to simple causes. We discuss the clinical picture of nasal and paranasal malignant peripheral nerve sheath tumor, its pathology, and its treatment.