CCHS is defined as alveolar hypoventilation in the absence of primary cardiac, lung, neuromuscular or focal CNS disease. Infants typically present in the newborn period with duskiness upon falling asleep. Though commonly diagnosed by a neonatologist, follow-up is typically by a pediatric pulmonologist or intensivist. We hypothesized that prior experience in the care of a child with CCHS would predict knowledge required to establish and treat this diagnosis. A survey was sent to 3,033 neonatologists, pediatric pulmonologists and intensivists affiliated with the American Academy of Pediatrics and/or the American Thoracic Society. The survey contained 27 questions to sample knowledge about diagnosis, characteristics, signs/symptoms, and therapeutic options for CCHS. Among 276 responses received by December 1,1997, 140 respondents (51%) had been involved in the care of a CCHS patient and represented 39% of responding neonatologists, 67% of responding pulmonologists, and 80% of responding intensivists. Results indicated that physicians who had been involved in the care of a CCHS patient were more likely to respond correctly to 100% of questions relating to diagnosis (all p values <0.0001, Chi Square analysis), 100% of questions relating to characteristics (all p values <0.0001), 100% of questions relating to signs/symptoms (all p values <0.0001), and 100% of question relating to therapeutic options (all p values <0.0001). Nonetheless, it remains disturbing that even among respondents with CCHS experience, the neonatologists as a whole answered only 49% of questions correctly, the pulmonologists 70%, and the intensivists 42%. These results demonstrate less than adequate knowledge among practitioners, regardless of experience. We propose increasing awareness and general knowledge of this condition to all pediatricians who might care for the CCHS patient through development and dissemination of a CCHS consensus statement and practice guidelines.