The goal of this study was to assess the quality of life for children with chronic intestinal pseudoobstruction. We used a retrospective chart review to identify children with congenital chronic intestinal pseudoobstruction, then a structured telephone interview with parents that included the Child Health Questionnaire to gather information about the current status and quality of life for each patient and family. Children with chronic intestinal pseudo-obstruction had less freedom from pain, depression, and anxiety than healthy children or children with juvenile rheumatoid arthritis (P < 0.05 for all three parameters). Parents of children with chronic intestinal pseudo-obstruction had poorer emotional status than parents of healthy children or children with juvenile rheumatoid arthritis. The time required for parents to care for children with chronic intestinal pseudo-obstruction was greater than the time required to care for healthy children or children with juvenile rheumatoid arthritis (P < 0.01). In conclusion, the quality of life for children with chronic intestinal pseudo-obstruction lags behind that of healthy children and children with another chronic illness. Appropriate treatment of chronic pain may improve the quality of life for children with chronic intestinal pseudo-obstruction and their families. Moreover, attention to reducing each family's burden of time and emotional distress may help them cope better with their chronically ill child.