A successful pregnancy in a patient with a 45,X/47,XXX karyotype who had premature menopause is described. Review of the literature revealed an additional 24 cases of fertility in women with gonadal dysgenesis associated with a 45,X chromosomal line. Their reproductive history showed a total of 58 pregnancies. However, abortion, stillbirth, and abnormal progeny, including five with chromosomal errors, were common. Therefore, such patients should be advised regarding their reduced fertility potential and the possibility of abnormal offspring.