Meningiomas are the most frequently occurring primary brain tumors in adults, representing almost one-third of all primary central nervous system tumors. Several factors have been suggested as an underlying cause in the development of meningiomas, such as ionizing radiation (therapeutic or other incidental exposure), hormonal factors, and genetic predisposition syndromes. Other established factors associated with meningiomas include age, female gender, and those from non-Hispanic Black backgrounds. Though the 2016 World Health Organization Classification of Brain Tumors largely preserves the existing grading scheme for organization of meningioma, there is increasing understanding of the molecular factors underlying the development of meningioma, some of which now form the basis for active clinical investigation. The mainstay of treatment has been the combination of radiation therapy and surgery, with a limited role for systemic therapy due to low efficacy, short duration of treatment response, and lack of uniform response criteria. Similar to other primary and metastatic brain tumors, immune-based therapies hold promise and are still under investigation.