OBJECTIVE: Premature ovarian insufficiency (POI), the loss of ovarian function before age 40, increases the risk of cardiovascular disease, low bone mineral density, dementia and psychological distress. Lifestyle interventions reduce chronic disease risk in other populations and, with hormone therapy, may improve health outcomes in POI. This review aims to identify the role of lifestyle, including diet and physical activity, in managing symptoms, improving quality of life (QoL) and preventing chronic disease in women with POI. The findings of this review informed the 2024 update of the ESHRE Evidence-Based POI Guideline. DESIGN: A systematic search was conducted in PubMed and Medline databases from January 2014 to February 2024. The review included randomized controlled trials and quasi-experimental trials that examined the impact of lifestyle interventions on women with POI. Outcomes included menopause symptoms, QoL, cardiovascular health and bone health. Risk of bias was assessed using Joanna Briggs Institute critical appraisal tool. RESULTS: The literature search yielded 890 citations, with one study meeting the inclusion criteria. Two additional studies from other guideline chapter searches were included, totalling three articles. Two studies involved cancer survivors and one included those with Turner syndrome. Limited evidence suggests lifestyle interventions, particularly physical activity, improve cardiovascular health and bone mineral density in women with POI. The effect of dietary supplementation was mixed. CONCLUSIONS: While a healthy lifestyle is proven to prevent chronic diseases and improve QoL in postmenopausal women, there is limited evidence specific to women with POI. Targeted studies are needed to determine the most effective interventions for addressing their heightened risks and unmet needs.