Within the head and neck region, neurilemmomas are rarely found in the nasal cavity or paranasal sinuses. A 70-year-old man presented with complaints of left-sided epiphora, rhinorrhea, epistaxis, and transient diplopia. Nasal endoscopy showed a large necrotic mass filling the left middle meatus. A computed tomography scan showed a large left ethmoid mass, with erosion of the medial wall of the orbit and the anterior cranial base. Multiple biopsies were nondiagnostic. Complete excision of the tumor was performed with endoscopic techniques. Small dural defects were repaired with a middle turbinate mucosal flap. Postoperatively, the patient was treated with nasal packing and a lumbar spinal drain. Final pathology showed a benign neurilemmoma. There has been no evidence of recurrence or cerebrospinal fluid leakage in follow-up. A review of the literature shows a similar presentation of patients with neurilemmomas of the paranasal sinuses. Bony destruction and intracranial extension is viewed. Lack of encapsulation and locally destructive growth in an otherwise histologically typical neurilemmoma should not suggest malignant potential. Many benign tumors of the paranasal sinuses with bone destruction may be removed by using endoscopic techniques.