AimThe aim of this paper is to present the evidence on the effectiveness of non‐surgical interventions to improve health and well‐being in women living with Mayer–Rokitansky–Kuster–Hauser (MRKH) syndrome.DesignSystematic review guided by Preferred Reporting Items for Systematic Reviews checklist.Data SourcesThe search was conducted between June and September 2022 across the following databases: CINAHL, EMBASE, Medline, PsycINFO and Cochrane. Trial registries (clinicaltrials. gov, World Health Organization International Clinical Trials Registry Platform (ICTRP), Cochrane Controlled Trials Register‐CCTR), Google scholar, dissertations, conference proceedings and reference lists of included studies were also searched. Corresponding authors, formal and informal MRKH groups were contacted to obtain any significant studies or reviews.Review MethodsEligible were only English‐language empirical studies of any time period. The review followed narrative synthesis.ResultsTwenty‐three studies were identified that fit the inclusion criteria which included 1540 MRKH syndrome affected women. Four studies were on psychological interventions (n = 85) and 19 studies (vaginal dilation therapy n = 897, coital dilation n = 57) focused on non‐surgical vaginal dilation as a measure to vaginal agenesis in MRKH syndrome.ConclusionsClearly, vaginal dilation is a viable initial treatment option for women with MRKH syndrome. There is limited evidence that ‘coital dilation’ is an effective method of dilation for vaginal agenesis. The literature, however, supports the need for psychological intervention to improve health and well‐being.ImpactWomen with MRKH syndrome who require dilation can receive guidance and support from their healthcare providers, particularly sexual and reproductive health nurses, clinical nurse specialists and gynaecologists. From the point of diagnosis, clinical psychologists should be involved. As much as feasible, family and partner support can be encouraged.Patient or Public ContributionNo patient or public contribution.