Currently, among all pathologies of the respiratory system, bronchiectasis receives special attention. This is due to its increasing incidence and the improvement of diagnostic methods. Williams – Campbell syndrome is a congenital form of bronchiectasis characterized by a defect or complete absence of bronchial wall cartilage in the subsegmental bronchi. In view of this, medical personnel should be familiar with the basics of differential diagnosis and treatment, as well as the prognosis of this disease. The aim of this paper is to present a clinical family case of Williams – Campbell syndrome. The father of the family was diagnosed with WCS at the age of 3 years by bronchography, and his daughter has all clinical signs of this disease today. To date, the question remains as how to diagnose WCS and monitor the patient’s health in the future. Conclusion. Physicians of all specialties should be informed about rare diseases.