Congenital laryngeal anomalies are relatively rare. However, they may present with life-threatening respiratory problems in the newborn period. Associated problems with phonation and swallowing may prevent a baby from thriving. Stridor is the most common presenting symptom of congenital laryngeal abnormalities. Often, it is associated with dysphagia, aspiration, and failure to thrive. Endoscopy is essential for evaluation and diagnosis in most cases. The differential diagnosis includes laryngeal cysts, atresia and stenosis, vocal fold immobility, and subglottic hemangiomas. In this article, the authors discuss in detail the evaluation and treatment for each condition.