Subglottic stenosis (SGS) is a congenital or acquired condition defined by a constriction of the upper airway that spans from slightly underneath the vocal folds to the lower edge of the cricoid cartilage. Congenital SGS is linked to a developmental anomaly during the tenth week of gestation and is commonly associated with other congenital head and neck abnormalities and syndromes. SGS is usually associated with iatrogenic injury or other causes of blunt or penetrating trauma, with 90% of cases attributable to prior or prolonged duration of intubation in both children and adults. Subglottic stenosis typically presents with biphasic stridor as the most common manifestation. In addition, patients may exhibit respiratory distress or recurrent upper respiratory infections, which cause inflammation that further narrows the subglottis. Evaluation in a patient with potential subglottic stenosis starts with a comprehensive medical history and physical assessment, including flexible fiber-optic laryngoscopy, focusing on ruling out airway blockages originating outside the larynx. When SGS is suspected, rigid direct laryngoscopy and bronchoscopy are required. The differential diagnosis typically involves croup, bacterial tracheitis, subglottic hemangioma, subglottic cysts, vocal cord paralysis, and complete tracheal rings. The treatment of SGS depends on the severity of the stenosis. Mild subglottic narrowing is often treated conservatively, while moderate-to-severe cases frequently necessitate surgery. Current treatment approaches include balloon dilation, endoscopic CO2 laser use, partial cricotracheal resection, laryngotracheal reconstruction, cricoid split, and tracheostomy.