Introduction: Balance training is important to improve balance and to decrease the risk of falls in patients with haemophilia (PWH). Aim: To analyse the current knowledge about the effectiveness of physical exercise on postural balance in PWH. Methods: A systematic search for clinical trials, published before November 2021, was conducted using the following electronic databases: PubMed/MEDLINE, Web of Science, Embase, Wiley Online Library and the Cochrane Central Register of Controlled Trials (CENTRAL). Two independent reviewers extracted the data and assessed the risk of bias. The certainty of the evidence was analyzed using GRADE. Results: A total of ten studies involving 304 patients were included. The studies performed strength and balance exercises, and some included flexibility, mobilization, and/or aerobic exercises in their training programs. Postural balance was evaluated using a force platform and the one leg stand test was the most frequently used. Only five exercise programs achieved a significant increase in balance (pre-post) with a strength, postural balance, flexibility, and aerobic intervention. In general, the quality rate of the risk of bias was fair but the overall quality of the evidence across the studies was very low. Conclusion: Five studies showed that there is a positive change in balance after the intervention. However, there is currently an unclear demonstration of evidence for the use of physical exercises to improve postural balance in people with haemophilia. Further studies with a higher methodological quality are needed.