OBJECTIVES: First, to compare the quality of life (QL) and perceived self-competence of children and adolescents with anorectal malformations or Hirschsprung disease with that of reference groups. Second, to identify predictors of QL. PATIENTS AND METHODS: A total of 491 patients with anorectal malformations or Hirschsprung disease were sent a questionnaire, which assessed QL (mental, physical), disease-specific functioning (defecation-related), perceived self-competence (self-esteem, athletic competencies, school attitude), and demographic characteristics (sex, age). The clinical characteristics (disease severity, presence of congenital anomalies) were extracted from medical records. RESULTS: More than 50% (316, 64%) of patients with anorectal malformations or Hirschsprung disease completed the questionnaire. On average, children and adolescents in both patient groups reported no differences in QL domains compared with the reference groups. However, standard deviations revealed considerable individual variation, indicating the presence of patients with high levels of QL as well as patients with low levels of QL. Children and adolescents in both patient groups reported psychosocial problems in all domains, compared with the reference groups. Females, older patients, and those with a severe form of the disease reported lower levels of perceived self-competence and global disease-specific functioning, which in turn predicted QL. CONCLUSIONS: Our results should alert clinicians to patients who are at risk for QL problems and may therefore be in need of extra care. Our findings illustrate the importance of both global disease-specific functioning and perceived psychosocial competencies for enhancing the QL of these patients.