Aim and background: Congenital tracheal stenosis is usually diagnosed during infancy because of respiratory distress. It presents as both inspiratory and expiratory stridor and is often associated with cardiopulmonary abnormalities. Undiagnosed tracheal stenosis without associated symptoms can lead to unanticipated difficult intubation and escalate to an airway emergency. Case description: We present a case of a 47-year-old female who was being evaluated for a renal transplant. A computerized tomography scan of the chest reported significant luminal compromise of the trachea at the level of the thyroid gland, with a maximum dimension of 7–8 mm in the sagittal plane. In the operating theatre, after confirming adequate face mask ventilation, a muscle relaxant (atracurium) was given as per body weight. Laryngoscopy was done using a size 3.5 McCoy, and the view according to the modified Cormack–Lehane was grade 2a. A size 6.0 mm internal diameter (ID) endotracheal tube, which has an outer diameter (OD) of 8.2 mm, was placed comfortably in the trachea. A size 6.5 tube (ID 6.5 mm, OD 8.9 mm) was found to be snugly fitting. The airway pressures were within the normal range during volume control ventilation. At the end of surgery, the trachea was extubated, and the patient was comfortable. Conclusion: There are no established airway management guidelines for such patients. In patients already diagnosed with tracheal stenosis, careful assessment and planning for intubation are essential for the smooth conduct of anesthesia. Clinical significance: A detailed history and careful inspection of available thoracic imaging can help in foreseeing unanticipated difficult intubations like tracheal stenosis.