OBJECTIVE To assess sexual education and sexual functioning in adolescents and young adults with spina bifida. METHODS Survey, inception cohort. METHODS The community. METHODS A cohort of adolescents and young adults (N=121; range, 15-35 y; 58% women) enrolled in a longitudinal pediatric database. METHODS Not applicable. METHODS Questions on sexual function, reproductive function, bladder and bowel continence, the Perceived Quality of Life Scale, and the Satisfaction With Life Scale. RESULTS Almost all adolescents and young adults with spina bifida in our study received sexual education at school, less at home, or by physicians. Twenty-five percent of men and 68% of women were informed about reproductive function by their physicians. Participants who reported that they smoked were 10 times more likely to report being sexually active and women were 2.3 times more likely to be sexually active than men. Hydrocephalus was a significant predictor of sexual activity among women but not men. Participants with urinary incontinence were less likely to be sexually active. Women without hydrocephalus were significantly more satisfied with life than women with hydrocephalus. CONCLUSIONS Adolescents and young adults with spina bifida in this sample were only slightly satisfied with life and sexual activity was only associated with life satisfaction among women. Dissatisfaction with life often leads to engagement in health-risk behaviors, which may, in part, account for the association between sexual activity and smoking behavior observed in these data. Further studies of health risk behaviors among youth with spina bifida are warranted and interventions aimed at reducing health risk behaviors among adolescents and young adults should specifically include spina bifida as a target group.