Objectives <p>To highlight the importance of a detailed evaluation of recurrent non-specific airway symptoms that can conceal dangerous vascular pathology in infants. This can avoid misdiagnosis, delayed management, and adverse outcomes.</p> Methods <p>Retrospective case series at a single academic tertiary care center from January 2008 to December 2022. Patient characteristics, symptoms, department at presentation, time between presentation and diagnosis, diagnostic methods, endoscopic findings, and patient comorbidities were collected.</p> Results <p>16 children met inclusion criteria. Fourteen (87.5%) presented with respiratory distress or stridor/noisy breathing to a clinic or the emergency department (ED). Two presented with feeding difficulty (2/16, 12.5%). In those presenting with respiratory distress, the mean age at presentation was 3.9 months. Fourteen (87.5%) underwent formal endoscopic airway evaluation for stridor. Four (25%) were diagnosed with a double aortic arch, 3/16 (18.75%) were diagnosed with an aberrant innominate artery, and 3/16 with a subglottic or oral hemangioma (18.75%). One (6.25%) presented with a right aortic arch, one (6.25%) an aberrant subclavian artery, and four (25%) had multiple anomalies. Surgical management was required in 12/16 (75%).</p> Conclusions <p>Airway vascular lesions are a rare but important cause of obstructive respiratory symptoms in children presenting to the ED in early infancy. The majority present before six months, with an average age of 3 months, at the time of diagnosis. Infants younger than six months of age presenting with recurrent episodes of stridor to an ED need an early detailed airway evaluation by a specialist to facilitate a timely diagnosis and optimize outcomes.</p>