This study examines the rate of utilization of mental health services in children and adolescents with 22q11DS relative to their remarkably high rate of psychiatric disorders and behavior problems. Seventy-two children and adolescents with 22q11DS were participants; their parents completed the Diagnostic Interview Schedule for Children (DISC) and the Child Behavior Checklist (CBCL). The results indicated that 22q11DS children and adolescents have higher rates of psychopathology than the general pediatric population, with ADHD and anxiety disorders being the most common. However, among youth with 22q11DS, those with psychopathology are often no more likely to receive either pharmacological or non-pharmacological mental health care than those without a given psychiatric diagnosis. Thus, although psychopathology is fairly common in this sample, many children with 22q11DS may not be receiving needed psychiatric care. These results have significant implications for these children and their families, as well as for the health care providers who treat them. In particular, the results may suggest a need for careful screening of psychiatric disorders that are likely to affect this population as well, as making appropriate treatment recommendations to remedy childhood mental health problems. Since these children face an extraordinarily high risk of psychoses in late adolescence/adulthood, treatment of childhood psychopathology could be crucial in mitigating the risk/consequences of major psychiatric illnesses in later life.