Background/Objectives: Few studies have adequately examined the unique issues of women with spina bifida as they enter their reproductive years. Most studies are anecdotal, retrospective case studies that contribute little to our understanding of the physiological effects of the disability on the reproductive system and, conversely, the effects of the reproductive endocrine changes on the woman's disability. The purpose of this article was to review previously published reports on the reproductive issues facing female adolescents and women and to ascertain the current knowledge so that future research needs can be established. Methods: Multiple MEDLINE searches were performed for publications from 1957 through the present pertaining to females/women with spina bifida and/or disabilities. Papers were then chosen if information was provided on menarche, menstruation and sexual maturation, gynecologic issues, sexual function, birth control, pregnancy outcomes, and menopause. These were then reviewed and classified according to the level of evidence (as defined by Ball et al). Results: More than 150 titles and abstracts were reviewed for inclusion. Of these, 75 papers met the guidelines for the subject content. Studies were stratified by level of evidence and included 1 study at level 3, 71 studies at level 4, and 3 studies at level 5. Conclusion: Little research has examined the reproductive issues of women with spina bifida and women with other neurologic disabilities. Future controlled prospective research studies are needed to examine issues related to puberty and sexual and gynecologic issues throughout the life span and pregnancy in these populations.