To systematically review the reported outcomes and complications of different treatment options for choroid plexus arteriovenous malformations (AVMs), specifically focusing on surgical resection and endovascular embolization.A systematic literature review was performed using a PubMed query for studies published between January 1975 and July 2021. All studies describing the clinical presentation, management, and outcome of confirmed choroid plexus AVM cases were included.A total of 20 studies were included in the final analysis. Of these, 18 were singlepatient case reports, one article contained two patients, and a single study was a cohort of 24 patients. Patient age ranged from one day to 61 years, with a mean of 31.8±20.4 years. Most choroid plexus AVMs were located in the lateral ventricles (14 patients, 70.0%), while there were four (20.0%) located in the third ventricle, and two in the fourth ventricle (10.0%). Almost all patients were treated with surgical resection (18 patients, 90%). In 14 patients (77.8%), complete resection of the AVM was achieved. A residual AVM was reported in one case (5.6%). Most patients were reported to have improved from their presentation status over time (14 patients, 70.0%). Presence or absence of long-term sequelae (e.g., neurologic deficits) were reported for 14 patients (70%). Eleven of these patients (78.6%) were reported to have no neurological sequelae.While data on choroid plexus AVMs remains limited, the available evidence suggests gross total resection of lesions in this location can be safely achieved with subsequent reduction in preoperative symptoms.