Purpose: The aim of this study was to identify factors associated with self-management behaviors in children with spina bifida (SB). The Individual and Family Self-Management Theory was used as a framework. Methods: This was a cross-sectional survey study of 112 elementary school children (7–12 years old) with SB who regularly attended scheduled follow-up appointments at the SB clinic of a children’s hospital in Seoul, Korea, and their parents. Demographics, condition severity, school adjustment, and family function were the contextual self-management factors, whereas self-management knowledge, self-efficacy, and social support were the process factors. Results: Age and condition severity were the contextual factors influencing self-management behaviors in children with SB; among the process factors, self-management knowledge and self-efficacy were statistically significant predictors. Conclusion: To improve self-management behaviors in children with SB, it is necessary to develop intervention programs that reflect their age and condition severity as contextual factors and aim to enhance self-management knowledge and self-efficacy as process factors.