ABSTRACT. Objective. Description of temperament profiles of children has largely been reported in typically developing populations. Children undergo individualized assessments of achievement (developmental/academic) and receive individualized interventions. In contrast, their individual behavioral styles are not evaluated as completely, if at all. For children with developmental disabilities, description of temperament characteristics can provide better understanding of the already complex child. This study describes temperament characteristics in a group of children with myelomeningocele and shunted hydrocephalus (MM/SH).Methods. A consecutive cohort study with historical control group measuring temperament characteristics was conducted at a tertiary-level, university affiliated, interdisciplinary spina bifida program. Analysis includes group comparisons. Primary caregivers of 46 children (age range: 5-12 years) with MM/SH completed age-appropriate Carey Temperament Scales questionnaires as a component of a larger developmental study. The Carey Temperament Scales comprise a series of behavioral rating instruments that assess 9 temperament characteristics: activity, adaptability, approach-withdrawal, mood, intensity, attention/persistence, distractibility, sensory threshold, and rhythmicity/predictability. The Carey questionnaires used in this study were (1) the Behavioral Style Questionnaire for children aged 5 to 7 years and (2) the Middle Childhood Temperament Questionnaire for those aged 8 to 12 years. Both questionnaires assess the same temperament characteristics; the contexts within which items were rated were designed to better reflect developmental levels. Children were excluded when there were comorbid diagnoses of attention-deficit/hyperactivity disorder or mental retardation.Results. One-sample normal tests (Bonferroni corrected) revealed that children within the MM/SH group differed significantly from the standardized population in 5 dimensions: (1) adaptability (less adaptable), (2) approach/withdrawal (poor first approach/greater withdrawal), (3) distractibility (more distractible), (4) attention/persistence (less attentive/persistent), and (5) predictability/rhythm (less predictable). Caregiver perceptions of having a difficult-to-manage child were significantly correlated with negative mood, more intensity of response, and less adaptability.Conclusions. Temperament profiles previously described in typically developing populations (eg, "easy" or "difficult child") were not prominent profiles in this group of children with MM/SH. A constellation of temperament characteristics not commonly recognized may place these children at risk for being contributors to and recipients of misunderstandings of social cues and have implications for successful learning within academic, home/community, and medical settings.