In the UK and Japan, midwives provide health services for women with concerns about a genetic condition or who are considering antenatal screening. In both countries, competences related to genetic health care have been devised but there is little evidence about midwifery competence in practice. A systematic literature review was undertaken to determine the extent to which midwives are achieving the genetic competences that are prescribed for their practice. English and Japanese literature from January 1999 to March 2009 was retrieved. Original studies or reviews, in which an aspect of midwifery practice was related to genetic competences, were eligible for inclusion. After a critical appraisal, six UK and five Japanese papers were eligible for inclusion. The findings indicated that midwives are not achieving the competences, nor are they confident about their genetics knowledge. Moreover, women are not being supported to make informed decisions regarding antenatal screening. We have confirmed that little research is being undertaken in both countries regarding competency achievement in practice. Changes to midwifery curricula and further continuing education are required to ensure that midwives are able to provide effective care regarding genetics.