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Methods: A case of a primary ectopic meningioma, arising within the right maxillary sinus, is presented. A review of English literature was also undertaken to determine the prevalence of these lesions in this specific anatomic location., Results: A total maxillectomy was performed without complications and the surgical defect was covered with an immediate obturator. The patient is well and without evidence of disease 3 1/2 years postoperatively. A review of the English literature revealed four additional reports of primary ectopic meningiomas that were limited to the maxillary sinus., Conclusions: Primary ectopic meningiomas of the maxillary sinus are uncommon lesions which have a good prognosis when a complete resection of the neoplasm can be performed.

Neurofibromatosis type II (NF-II) has been traditionally referred to as "acoustic" neurofibromatosis and is not known to be genetically distinct from classic von Recklinghausen's disease (NF-I). Neurofibromatosis type II is due to a lesion on chromosome 22q, while von Recklinghausen's neurofibromatosis is from a defect on chromosome 17. The approximate incidence of NF-II is one in 50,000, with bilateral acoustic neuromas occurring in over 90% of those with the abnormal gene. We studied a 17-year-old boy with NF-II who presented with bilateral acoustic neuromas and concomitant primary nasopharyngeal meningioma. Less than 30 nasopharyngeal meningiomas have been documented, and the presence of both tumor types in the same individual is unique in the literature.