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Introduction Unruptured brain arteriovenous malformations (AVMs) can rarely present with hydrocephalus, however there is limited data guiding the treatment of these patients. A systematic review with institutional case report was performed to evaluate safety and efficacy of treatment.Methods A systematic review was conducted following the Preferred Reporting Items for Systematic Reviews and Meta Analyses guidelines from January 2000 to March 2026. All studies reporting unruptured brain AVMs causing hydrocephalus were included.Results 19 articles met inclusion criteria comprising 33 patients who presented with hydrocephalus, including our own. The average age at presentation was 35.9 ± 21.6 years old and 54.5% of patients were male. AVMs were most commonly located at the basal ganglia, cerebellum or midbrain and the average size was 3.4 ± 1.7 cm. 23 patients (70%) had obstructive hydrocephalus, due to mechanical obstruction of a draining vein. 12 patients (30%) had impaired CSF fluid resorption due to venous congestion. 19 patients (57%) underwent CSF diversion with either shunting or ETV. 21 patients had additional treatment of their AVM (64%) with 11 patients undergoing only endovascular embolization (33%), 6 patients undergoing only radiosurgery (18%) and 5 patients undergoing multimodality treatment (15%). 6 patients had a complication related to CSF diversion (18%), including 1 AVM rupture. 1 patient had a complication related to AVM treatment (3%). While follow up is limited, most patients did well in the short term.Conclusion Unruptured brain AVMs rarely present with hydrocephalus, most commonly due to mechanical obstruction. Treatment of hydrocephalus alone or with associated treatment of the AVM is effective, however, there remains a low risk of AVM rupture with CSF diversion.Disclosures A. Gong: None. C. Dewar: None. S. Salas-Vega: None. E. Geist: None. V. Padmanaban: None.