The aim of this study was to describe marital status, sexual history, and sexual functioning in a group of women with Turner syndrome, and to compare the results with general Swedish population data. The sample consists of 57 women over 18 years of age. Data were collected from an interview, and using two self-report questionnaires: the McCoy Sexual Rating Scale and the Relationship Rating Scale (RS). Compared to population data, the women with Turner syndrome were less likely to have a partner and had had their sexual debut later. Single women differed more from the general population than did women with a partner, regarding sexual desire and sexual activity. Several women with a partner reported sexual problems, but unanimously reported being satisfied with their sex life and partner relationship. The level of sexual desire in women with Turner syndrome is discussed in relation to Levine's model of human sexual desire, where psychological and social motivational factors are considered in addition to a biologically based sexual drive (Levine, 1992).