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BACKGROUND: Bile acid diarrhoea, a common cause of diarrhoea-predominant irritable bowel syndrome, has a similar prevalence to coeliac disease and inflammatory bowel diseases. The mainstay of treatment is bile acid sequestrants (BAS). The effectiveness of using bile acid-targeted therapies remains unclear. AIM: To compare BAS with placebo for bile acid diarrhoea. METHODS: We conducted a systematic review and meta-analysis of randomised controlled trials comparing BAS to placebo using a comprehensive search of databases from inception to 8/1/2023. Inclusion required unequivocal diagnosis using serum or faecal biochemical parameters, or 75SeHCAT. Co-primary endpoints were ≥ 30% improvement in bowel movement consistency and frequency averaged over 7 days. Adverse effects were also summarised. RESULTS: Database search resulted in 1152 citations; 1 additional study was added. Ultimately, 6 trials (5 parallel arm, 1 crossover) involving 182 participants evaluated the effectiveness of BAS over 1-8 weeks. Participants were between the third and seventh decades of life and mostly female. The risk of bias was low except in one parallel and one crossover trial. Meta-analysis of parallel-design trials of sequestrants only showed a clinically significant reduction in stool consistency and frequency with pooled relative risks of 1.50 (95% CI: 1.14-1.96) and 2.80 (95% CI: 1.68-4.67), respectively. There was no observable heterogeneity (I2 = 0%), and the certainty of evidence was moderate. There was no statistically significant difference in adverse effects. CONCLUSIONS: This study quantifies the significant and rapid improvement in both stool consistency and frequency with BAS in patients with objective diagnosis of bile acid diarrhoea. Sequestrants also appear well tolerated.