OBJECTIVES: Children with spina bifida report both chronic and acute pain caused by both their condition and the treatments they undergo regularly. This research provides a description of the impact of this pain on their quality of life. METHODS: A sample of 68 children (56% female) between the ages of 8 and 19 completed the Varni/Thompson Pediatric Pain Questionnaire, a supplementary questionnaire on pain, the Children’s Depression Inventory, the Nowicki-Strickland Locus of Control Scale for Children, and a pediatric measure of health related quality of life. RESULTS: Health related quality of life was shown to be systematically low in this group as compared with a reference sample of chronically ill children. It was negatively impacted by high reported frequency of pain and high ratings of current pain. Both pain and low quality of life were strongly associated with Children’s Depression Inventory scores. Locus of control scores was not associated with quality of life or reported pain. CONCLUSION: The unmanaged pain in children with spina bifida can have a substantial negative impact on quality of life. Better treatment and surveillance of pain and depression symptoms may significantly improve quality of life.