目的探讨和总结口腔颌面神经鞘瘤的临床特点、鉴别诊断和治疗。本文报道1例46岁女性上颌牙龈神经鞘瘤患者。分析其临床特点、病理特点、鉴别诊断和治疗。检索1986年~2017年国内外期刊全文数据库文献，中国医院知识库和PubMed数据库中1986年~2017年有关口腔颌面部神经纤维瘤的报道39篇，共405例患者。牙龈粘膜瘤23例，国外文献17例，国内文献仅6例。牙龈神经鞘瘤发病率极低，好发年龄与其他部位相似，以中青年为主，无明显的性别倾向。大约 25-45% 的神经鞘瘤出现在头颈部，很少见于口腔（仅 1%）。口腔最常见的内部位置是舌头，其次是口底、颊粘膜、上颚、牙龈和嘴唇。神经鞘瘤是生长缓慢的良性肿瘤，在牙龈中很少见。牙龈神经鞘瘤通常为单发，临床表现多为无痛性牙龈肿块、牙齿松动、移位，无周围骨质改变和区域淋巴结转移。该肿瘤根据临床表现诊断较困难，病理诊断仍是牙龈神经鞘瘤诊断的依据。迄今为止，手术切除是该病的首选治疗方法，预后良好。© 2023。BioMed Central Ltd.，Springer Nature 旗下公司。

To explore and summarize the clinical features, differential diagnosis and treatment of the oral maxillofacial schwandoma.This is a report of a case of a 46-year-old female patients with neurolemmoma in the maxillary gingiva. The clinical features, pathological features, differential diagnosis and treatment were analyzed. Literature review was conducted in search of domestic and overseas journal full-text database from 1986 ~ 2017. 39 reports on the oral and maxillofacial Neurolemmoma from 1986 to 2017 in the database of China hospital knowledge database and the PubMed database, there were 405 patients. There were 23 cases of gingival mucosa, 17 in foreign literature and only 6 in the domestic literature.The incidence of gingival Neurolemmoma is extremely low, the predilection age is similar to other parts, it is middle-aged and young, and there is no obvious gender tendency. About 25-45% of schwannomas are found in the head and neck, and rarely in the mouth (only 1%). The most common internal location of the mouth is the tongue, followed by the floor of the mouth, buccal mucosa, palate, gums, and lips. Schwannomas are slow-growing benign tumors that are rare in the gums. Gingival schwannoma is usually a single occurrence, and the clinical manifestations are mostly painless gum mass, tooth loosening and displacement, without peripheral bone changes and regional lymph node metastasis. It is difficult to diagnose this tumor according to clinical manifestations, and pathological diagnosis is still the basis for the diagnosis of gingival schwannoma. So far, surgical resection is the preferred treatment for this disease, and the prognosis is good.© 2023. BioMed Central Ltd., part of Springer Nature.