The rate of congenital malformations of the larynx is challenging to assess. This is probably accounted for by the fact that these diseases occur more commonly than they are diagnosed. This paper describes a case report of congenital laryngeal stenosis, which was a diagnosis of exclusion since this congenital malformation is very rare in neonatal and pediatric practice. After a preliminary diagnosis in the maternity hospital, a newborn was transferred to Krasnoyarsk’s large pediatric hospital for examinations and treatment. Clinical diagnosis was verified by laryngotracheoscopy and CT scan of the larynx and trachea. The newborn’s condition was severe for 28 days. He stayed in the intensive care unit and was on a ventilator and oxygen. Tracheal intubation followed by extubation (performed three times) has no effect due to the increase in respiratory failure. Newborn’s condition improved only after palliative tracheostomy. The child was discharged in satisfactory condition with recommendations on tracheostoma care. Management decision on the surgical technique to address this malformation will be taken later. KEYWORDS: primary congenital tracheal stenosis, children, clinical presentations, diagnosis. FOR CITATION: Gol’tsman E.V., Kuchma O.A., Bondarenko E.N., Maiseenko D.A. Congenital tracheal stenosis: case report. Russian Journal of Woman and Child Health. 2021;4(3):298–301 (in Russ.). DOI: 10.32364/2618-8430-2021-4-3-298-301.