This article has attempted to describe an approach to the assessment and management of the child with choanal atresia whose airway abnormality is one component of a multiple anomaly condition (CHARGE association). The risks to the child with CHARGE association exceed those of any one of his or her anomalies, and the clinician must assess the child deliberately, realizing that morbidity may be increased if any of the child's other systemic anomalies are given a secondary role in long-term management. The CHARGE association baby is an atypical choanal atresia patient, as he or she is an atypical cardiac or hearing-impaired patient; for each child there may be a logical approach to prioritizing such issues as nutrition, airway management, and cardiovascular support. Unlike known "syndromes" in which natural history may be definable, the child with CHARGE association depends considerably on the effects of various interventions to determine outcome. Some basic principles useful in guiding these interventions have been presented to assist the clinician in this process.