The etiology of fourth ventricle ventricular meningiomas is unknown and may be related to genetic mutations, along with potential risk factors such as electromagnetic radiation. Fourth ventricle ventricular meningiomas are ventricular meningiomas originating in the IV ventricle, mainly RELA fusion gene-positive ventricular meningiomas. The exact cause of the disease is unknown and may be related to a genetic mutation, and type 2 neurofibromatosis increases the risk of developing ventricular meningiomas. There are also a number of possible underlying risk factors, such as electromagnetic radiation, neurologic carcinogens, allergic diseases, and viral infections. Tumors often originate in the fourth ventricle, are often associated with hydrocephalus, and can metastasize through the cerebrospinal fluid, resulting in a poor prognosis. It is recommended that patients with fourth ventricle ventricular meningioma should seek medical treatment in time and under the guidance of doctors, so as to avoid delaying the condition and causing serious consequences.