Purpose: To assess quality of life outcomes specific to the new population of children with congenital intestinal pseudo-obstruction (CIP) who are living longer. Methods: Antroduodenal manometry confirmed disordered motility in 36 children (16 male, age 1 y-20 y, X=9y). Via chart audits and telephone interviews, we obtained information about disease status, symptoms, and feeding methods. We used the Child Health Questionnaire to measure physical, social, and emotional functioning of the child and family. Results: We diagnosed 12 with myopathy, 23 with neuropathy and 1 unclassified. Children with myopathy (10 of 11) were more likely than those with neuropathy (5 of 23) to have urinary bladder involvement (p<.001). Malrotation was found in 4 of 10 children with myopathy and 6 of 23 with neuropathy. Current symptoms were constipation (42%), diarrhea (42%), and vomiting (48%). Compared to early life, there was a decrease in vomiting (p<.01), failure to gain weight (p<.001), and urinary tract infections (p<.05). Parenteral nutrition was required by 36% and tube feeding by 22%. Two-thirds eat >10% of their caloric requirement. Comparing QOL to healthy children on a scale from 0 to 100, the CIP children had lower levels of physical functioning (healthy, x=96; CIP, x=82). Self-esteem (healthy, x=80; CIP, x=76), mental health (healthy, x=78; CIP, x=73), and behavior (healthy, x=74; CIP, x=73) were comparable. CIP children scored worse than children with juvenile rheumatoid arthritis (x=62) or asthma (x=78) in bodily pain (x=50). There was a profound impact on parents' emotional status (JRA, x=72; asthma x=74; CIP, x=65), and time required for health management (JRA, x=86; asthma, x=83; CIP, x=31). Discussion: With supportive care children with CIP survive and grow. The QOL for a child with CIP is similar to healthy children and children with other chronic illnesses in many ways. Despite this positive outcome, we identified two areas of relatively poor outcome: 1) chronic visceral pain and 2) QOL of parents who suffer emotional stress and time loss related to CIP.