Extracranial metastases from meningioma are extremely rare with an incidence of <1% and their prognosis is poor. Moreover, there is currently no gold standard for their treatment and, therefore, the decision making is strictly depending upon multidisciplinary discussion. In this re-port, we describe the case of a 73-year-old patient who was diagnosed with a solitary lung metas-tasis over 20 years after initial treatment for a low-grade meningioma. Molecular characterization of this metastasis was performed by Oncomine Comprehensive Assay Plus recording multiple func-tional mutations on b2microglobulin and ATM genes. A short overview of the literature is also reported. To our knowledge, no previous reports exist on single pulmonary metastasis from low-grade meningioma occurring more than 20 years after diagnosis.