A 45-year-old man, afflicted with von Recklinghausen's disease, with symptomatic schwannomas and plexiform neurofibromas involving the right maxillary antrum, a rare site for peripheral nerve tumors, is presented. After a literature review, 15 cases were considered adequate for a clinicopathologic study. Twelve schwannomas, 2 plexiform neurofibromas, 2 neurofibromas, and 1 probable malignant schwannoma were included. Epistaxis was common in tumors of the ethmoid sinus and nasal fossae, while pain was related to lesions of the maxillary sinus. Two benign schwannomas recurred and were cured by surgery alone. The importance of early diagnosis and thorough surgical excisions is emphasized, especially for those tumors associated with von Recklinghausen's disease. The difficulties involved in the diagnosis of primary malignant peripheral nerve tumors are discussed.