A review was undertaken of the range of possible congenital lesions of the major airways and adjacent tissues that may cause critical compromise of luminal diameter with resultant respiratory arrest from airway occlusion. Lesions included micrognathic syndromes, macroglossia, choanal atresia and stenosis, tumors and choristomas, posterior lingual masses, laryngeal atresia and stenosis, laryngeal webs, laryngeal cysts and laryngoceles, laryngomalacia, tracheomalacia, and bronchomalacia. An autopsy approach to possible congenital obstructive lesions of the upper airway requires: (1) review of the clinical and family histories looking specifically at the nature of the terminal episode; (2) external examination looking for dysmorphic syndromes with mandibular or mid-facial hypoplasia; and (3) internal examination with layer dissection of the soft tissues of the neck and en bloc removal of the upper aerodigestive tract, with photographic recording and histological sampling.