The purpose of this study was to (a) examine the occurrence of psychiatric symptomatology in children and adolescents with spina bifida, (b) investigate the relationship between psychiatric features and aspects of disability, and (c) explore the impact of spina bifida and psychiatric status on family functioning. Fifty-four children and adolescents ages 6 to 18 years (M = 12.94, SD = 3.59) were examined. Parents completed the Child Symptom Inventory (CSI) and the Family Assessment Device (FAD). Using the CSI, a psychiatric diagnostic screen, 43% of the sample obtained one, and 13% obtained two or more screening cutoff scores reflective of psychiatric diagnoses. The two most prevalent diagnostic categories were Attention-Deficit/Hyperactivity Disorder (33%) and Oppositional Defiant Disorder (13%). The sample as a whole exhibited elevated levels of clinical symptoms, with internalizing symptoms more prominent than externalizing symptoms. No differences in diagnostic categories or overall symptomatology were found based on age, gender, ambulation status, or lesion level. Overall symptom counts were positively correlated with scales on the FAD reflecting problematic family functioning (.42–.65). Results suggest that psychiatric symptomatology occurs at a high rate in children and youth with spina bifida. Although ADHD was the modal diagnostic category, the sample as a whole exhibited extensive psychiatric symptoms independent of specific diagnostic categories. Psychiatric symptoms were also associated with increased problematic functioning in families.