We reviewed the diagnosis, complications and treatment of congenital laryngeal stridor (CLS), in 97 patients who consulted our clinic between 1991 and 2001. The 97 patients were diagnosed with laryngeal malacia (32%), vocal cord paralysis and laryngeal stenosis (22%), a neoplastic disease like hemagioma and papilloma (11%), or cystic disease (7%). The cases with vocal cord paralysis, laryngeal stenosis or laryngeal cysts were usually diagnosed within 2 months of birth based on severe dyspnea. Two of the 31 cases of laryngeal malacia and 2 of the 22 cases of vocal cord paralysis were associated with neuromuscular disorders. Three patients suffered from vocal cord paralysis complicated by laryngeal stenosis. Thirty-three of the 97 cases required a tracheostomy; these 33 cases included the one case of laryngeal papilloma (100%), 9 of the 10 cases of hemangioma (90%), and 18 of the 24 cases of laryngeal stenosis (75%). Since any disorders of the upper airway can potentially induce stridor, establishing an accurate diagnosis is sometimes difficult when stridor is the only presenting symptom. Hence, information on associated symptoms and the past history of the subject is particularly important for an accurate diagnosis. In addition, decisions regarding the course of treatment course require adequate consideration of possible complications.