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Stridor is a high-pitched respiratory sound that signals upper airway obstruction. It can be encountered by clinicians in a variety of clinical settings and requires a team-based, interdisciplinary approach. Early recognition is crucial, as the differential diagnosis can be broad, and causes range from benign to life-threatening. This article reviews the most commonly encountered causes of chronic congenital stridor in infants, focusing on the diagnostic approach, pathophysiology, clinical presentation, and management strategies.