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Objective This study was designed to examine whether executive functions and parenting behaviors (acceptance, behavioral control, and psychological control) are associated with medical adherence and autonomy among preadolescents and adolescents with spina bifida (SB). Methods Questionnaire and observational data were collected from a sample of 8–15 year olds with SB (N = 140) and their mothers, fathers, and teachers. Youth also completed neuropsychological testing. Results Youth with SB demonstrated impairment on measures of executive functions, based on questionnaire and test data. Executive functions (questionnaire data only) and parenting behaviors were associated with medical adherence, but only executive functions (test data only) were associated with medical autonomy. Analyses also suggest that maternal and paternal behavioral control and paternal psychological control moderate relations between executive functions and adherence. Conclusions Interventions that target executive functions and parenting behaviors may facilitate positive health care behavior outcomes among youth with SB.