To systematically review the evidence for balance impairment and effectiveness of interventions on balance in people with chronic obstructive pulmonary disease (COPD). Four electronic databases (Scopus, CINAHL, PubMed, Cochrane Library databases) were searched from inception until June 30, 2019. Two reviewers independently searched with keywords focusing on COPD, postural control, and exercise. Cross-sectional studies related to balance and randomized controlled trials (RCTs) related to the effectiveness of exercise intervention on balance outcomes were included. Two reviewers independently extracted data of balance impairment on participants, exercise training on balance outcome measures. Methodological quality of cross-sectional studies was assessed using the National Institutes of Health (NIH) Quality Assessment Tool. Methodological quality of RCTs was assessed using the Physiotherapy Evidence Database (PEDro) scale and bias was analyzed using the Cochrane risk of bias. A narrative review with descriptive synthesis was used. Fifteen cross-sectional studies and 4 RCTs met the final inclusion criteria. The quality of 10 cross-sectional studies were rated as moderate to high (NIH score≥7). Most studies demonstrated impaired balance in people with COPD than in people without COPD. The quality of all included RCTs was good to excellent (PEDro score=6-9). One RCT had a low risk of bias. Generally, pulmonary rehabilitation program combined with balance training, Tai Chi, and cycling exercise showed significant improvement in balance in people with COPD. Impaired balance is evident in people with COPD. Available RCTs suggest that exercise interventions may improve balance performance in COPD patients. However, more research on the effect of exercise interventions on balance in COPD patients is still required. Copyright © 2020 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.