AIMS: To systematically identify the application of Acceptance and Commitment Therapy among parents of children with chronic health conditions and determine its effectiveness in parental psychological flexibility, psychological distress and parenting behaviour. DESIGN: Systematic review. DATA SOURCES: Nine databases (i.e. MEDLINE, PubMed, Embase, Cochrane Library, CINAHL, PsychINFO, Web of Science, China National Knowledge Infrastructure and WanFang Data) were systematically searched from inception to October 2019. REVIEW METHODS: Quality of studies was appraised by using the Joanna Briggs Institute critical appraisal checklist. Findings were synthesized narratively. This work was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis statement. RESULTS: Eight studies involving 485 parents were included. Results indicated that Acceptance and Commitment Therapy significantly improved parental psychological flexibility and reduced psychological distress compared with usual care and waitlist, but was not significantly different from active treatments. Limited studies have provided very preliminary evidence that Acceptance and Commitment Therapy can significantly improve dysfunctional parenting behaviour than usual care and waitlist. High attrition rate at follow-up made the overall confidence of maintained effect relatively low. CONCLUSION: This review provides preliminary evidence that Acceptance and Commitment Therapy is beneficial for improving psychological flexibility, psychological distress and parenting behaviour among parents of children with chronic health conditions. Future studies with rigorous designs and large sample sizes are warranted to verify the evidence and explore its long-term efficacy. IMPACT: Acceptance and Commitment Therapy has been increasingly applied to parents of children with chronic health conditions. This review provides positive evidence of its effects on psychological and behavioural outcomes among these parents. This work will help healthcare professionals and researchers with their practice and further research