Aims The evidence-based recommendation for the use of systemic bevacizumab to treat refractory epistaxis in hereditary haemorrhagic telangiectasia (HHT) is based on limited and historical data. An updated synthesis of the available literature is warranted to evaluate the use of systemic bevacizumab for HHT-related epistaxis, including its effect on treatment outcomes and adverse effects. Methodology A systematic review and meta-analysis were conducted using PRISMA guidelines. A search was conducted using 3 databases up to September 2024 for studies assessing the effect of systemic bevacizumab use on epistaxis outcomes in HHT. Outcome measures included Epistaxis Severity Score (ESS), haemoglobin, red blood cell (RBC) and iron transfusion requirements and adverse effects. Random effects meta-analysis was performed for change in ESS (ΔESS) and change in haemoglobin (ΔHb). Literature quality was assessed using the Joanne Briggs Institute critical appraisal tools. Results Ten studies with 225 total patients were included. Systemic bevacizumab was associated with a significant post-treatment reduction in mean ESS of -3.33 (95% CI -3.62 to -3.03) and a significant increase in mean Hb of 2.38 g/dL (95% CI 1.45–3.30) compared to pre-treatment. All cohort studies found significant reduction in RBC and iron transfusions following systemic bevacizumab treatment. The most frequently reported adverse effect of systemic bevacizumab was hypertension. Conclusion Systemic bevacizumab is associated with significant improvements in epistaxis severity, haemoglobin and transfusion requirements in HHT-related epistaxis. Adequately powered studies are required to strengthen this finding. Patient selection criteria, standardised maintenance dosing and long-term treatment data require further study.