Defective or incomplete separation by the tracheoesophageal septum is one of the most frequent congenital anomalies producing tracheoesophageal fistula (TEF). Anatomic variations that can influence the planning and success of a tracheotomy may be broadly grouped into extrinsic and intrinsic causes. The etiology of the nasal obstruction may include an anterior congenital nasal pyriform aperture stenosis (which is rare), a tumor, or choanal atresia. The most common congenital malformation of the esophagus is esophageal atresia, with or without TEF. Congenital tracheal stenosis may be associated with congenital heart disease, TEF, and skeletal abnormalities. Treatments include tracheoplasty, resection with reanastomosis and stenting. Direct trauma can result in cartilaginous damage and occlusion of the airway lumen by hemorrhage, edema, granulation tissue, scarring, or structural collapse. Congenital tracheal webs are rare presenting with stridor, wheezing, and recurrent respiratory infections. The most common pediatric tracheobronchial tumors include hemangioma, bronchial carcinoid, and papillomatosis.