This article is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License (CC BY-NC-ND) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes as well as any distribution of modified material requires written permission. What Is It about? • Respiratory distress is the most common reason for admission to neonatal intensive care units for both term and preterm infants. As it might be a life-threatening condition, it is crucial to diagnose and treat it quickly. Congenital airway anomalies are a relatively rare cause of respiratory distress. They should be considered when the patient does not respond to routine interventions and when intubation is difficult. Congenital laryngeal stenosis may exist as an isolated entity or in association with other congenital malformations. In this paper, we present the case of an infant with congenital laryngeal stenosis and tetralogy of Fallot.