OBJECTIVE: Epley maneuver is the most effective treatment for benign paroxysmal positional vertigo (BPPV). Betahistine has been shown to be effective in treating BPPV. This systematic review aims to compare the effectiveness of Epley maneuver with betahistine to Epley maneuver alone in reducing residual dizziness in BPPV. DATA SOURCES: Medline, Embase, and CENTRAL. METHODS: We included randomized controlled trials (RCTs) that compared Epley maneuver with betahistine to Epley maneuver alone for treating BPPV. We evaluated the effectiveness of this intervention using Dizziness Handicap Inventory (DHI), Visual Analog Scale (VAS) for vertigo, and provocation maneuvers. We used the standardized mean difference (SMD) for continuous outcomes and the odds ratio (OR) for the dichotomous outcomes. RESULTS: A total of eight RCTs that enrolled 516 participants were deemed eligible. Administration of betahistine with Epley maneuver showed no clinically significant difference over Epley maneuver alone in DHI score (SMD: -0.11, 95% confidence interval [CI], -0.57 to 0.34, CONCLUSION: Combining betahistine with Epley maneuver could improve the outcomes of BPPV in the long term. However, clinical trials with longer follow-up periods are needed to unravel its efficacy.