Intramedullary schwannomas (IMS) represent exceptional rare pathologies. They commonly present as solitary lesions; only five cases of multiple IMS have been described so far. Here, we report the sixth case of a woman with multiple IMS. Additionally, we performed the first complete systematic review of the literature for all cases reporting IMS. We performed a systematic review of the literature in PubMed, EMBASE and Cochrane Central Register of Controlled (CENTRAL) to retrieve all relevant studies and case reports on IMS. In a second step, we analysed all reported studies with respect to additional cases, which were not identified through the database search. Studies published in other languages than English were included. One hundred nineteen studies including 165 reported cases were included. In only five cases, the patients harboured more than one IMS. Gender ratio showed a ratio of nearly 3:2 (male:female); mean age of disease presentation was 40.2 years; 11 patients suffered from neurofibromatosis (NF) type 1 or 2 (6.6%). IMS are rare. Our first systematic review on this pathology revealed 166 cases, including the here reported case of multiple IMS. Our review offers a basis for further investigation on this disease.

髓内神经鞘瘤 (IMS) 代表异常罕见的病理。它们通常表现为孤立性病变；到目前为止，只描述了 5 种多重 IMS 的情况。在这里，我们报告了第六例患有多个 IMS 的女性。此外，我们对所有报告 IMS 的病例进行了第一次完整的文献系统审查。我们对 PubMed、EMBASE 和 Cochrane Central Register of Controlled (CENTRAL) 中的文献进行了系统回顾，以检索关于 IMS 的所有相关研究和病例报告。在第二步中，我们分析了所有关于其他病例的报告研究，这些病例未通过数据库搜索确定。以英语以外的其他语言发表的研究也包括在内。包括 165 个报告病例在内的 119 项研究被纳入。仅在五种情况下，患者携带不止一个 IMS。男女比例接近3：2（男：女）；平均发病年龄为 40.2 岁；11 名患者患有神经纤维瘤病 (NF) 1 型或 2 型 (6.6%)。IMS 很少见。我们对这种病理学的第一次系统评价揭示了 166 例病例，包括此处报告的多个 IMS 病例。我们的审查为进一步调查这种疾病提供了基础。