Schwannomas, also known as neurilemomas or neurilemmomas, are relatively uncommon, slow-growing benign tumors. Whereas, about one-third of all extracranial Schwannomas are found in the head and neck region, a few intraoral Schwannomas are reported in the literature. This article contributes to a review regarding the current literature and the report of a rare case. The literature searches were performed using the National Library of Medicine. Keywords used in the search were: schwannoma or neurilemmoma and intraoral. The literature search revealed 16,906 reports containing the word schwannoma; however, only 1,117 articles described this tumor entity in the “head and neck” region. The search item intraoral, in addition to schwannoma or neurilemmoma, were found in only 29 reports. In most cases, intraoral schwannomas are benign, slowly growing tumors. The treatment of choice is surgical excision. However, malignant schwannomas can also occur, and need a radical resection and a dissection of the regional lymph nodes.
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