Hydrocephalus (HC) can be associated with vestibular schwannoma (VS) at presentation. Although spontaneous resolution of HC after VS removal is reported, first-line treatment is varied including preoperative ventriculoperitoneal (VP) shunt, external ventricular drainage (EVD), or lumbar drainage (LD). We performed a systematic review to clarify optimal management of HC associated with VS at presentation, as well as characteristics of patients with initial and persistent HC after VS removal, and prevalence of HC associated with VS. Fourteen studies were included. Patients were grouped according to the timing of HC treatment. The overall rate of VP shunts was 19.4%. Among patients who received VS removal as first-line treatment, 6.9% underwent permanent shunts. In a subgroup of 132 patients (studies with no-aggregate data), t test analysis for mean tumor size (P = 0.02) and mean CSF protein level (P < 0.001) demonstrated statistically significant differences between patients with resolved HC (3.48 cm and 201 mg/dL) and patients with persistent HC (2.46 cm and 76.8 mg/dL) after VS resection. Transient treatment of HC using EVD or LD further resolved the HC in 87.5% and 82.9% of patients, respectively, before and after VS removal. The overall prevalence of HC associated with VS in a population of 2336 patients was 9.3%. Schwannoma removal as first-line treatment is justified by its low rate of persistent HC requiring VP shunt (roughly 7%). Patients with smaller VS and lower CSF proteins present higher risk of persistent HC after schwannoma removal. Temporary treatment of HC contributes to its resolution, both before and after VS removal.