Background: Congenital tracheal stenosis is defined as the narrowing of the airway lumen due to the abnormal formation of complete tracheal cartilage rings. It may present with different clinical pictures, depending on the range and extent of stenosis. We present two cases of congenital tracheal stenosis, with a different range of severity and, consequently, different treatment approaches. Case Presentation: We describe the case of a 3-month-old infant with breathing noise that worsened particularly when crying and after nutrition. He presented a congenital funnel-shaped tracheal stenosis with an associated tracheal bronchus, which was treated endoscopically with an endobronchial stent. The second presented case is of a newborn who presented severe respiratory distress and inspiratory stridor in the first hours of life. A congenital cylindrical tracheal stenosis was diagnosed and it was treated surgically through slide tracheoplasty. Discussion: These two cases showed us that congenital tracheal stenosis can present with a very variable clinical picture. Thus, according to clinical presentation, different treatments can be considered. Conclusion: We underline the importance of considering congenital tracheal stenosis in the differential diagnosis of prolonged wheezing and recurrent airway infections.