Bronchiectasis (BR) is a respiratory disease, anatomically defined by abnormal and progressive bronchial dilatation, clinically manifested by chronic cough with sputum production, and recurrent respiratory infections. Despite many studies conducted over the years, using modern technologies and methods up to 40% of BR are idiopathic. We present a clinical case of a young man, with idiopathic BR, frequent pulmonary infections since childhood, associated with progressive hepatic impairment. A 36-year-old male, non-smoker, with congenital heart malformation (persistent foramen ovale), viral cirrhosis B ChildPugh C, with recurrent respiratory infections, in 2017 was diagnosed with bilateral, apical, varicose bronchiectasis. Due to this distribution of BR and to the onset of disease from childhood, a cystic fibrosis or α1-antitrypsin deficiency was suspected, that further were not confirmed (normal sweat test and serum α1-antitrypsin level). Diffuse ground glass opacity on HRCT, dilated pulmonary arteries (MPA 39,6 mm, RPA 28,3 mm, LPA 30 mm) with confirmed pulmonary hypertension on echocardiography (systolic pressure of pulmonary artery is 60 mmHg), with ”sausage fingers” – are highly suggestive for a collagenosis. Evaluated autoantibodies (anti-CCP, ANA, Scl-70) established increased level only of anti-CCP (40,8 U/ml). He has at least 2 exacerbations of BR per year, marked by signs of respiratory failure (dyspnea mMRC IV, SaO2˂85% without O2 support, acrocyanosis), cough with purulent sputum 30 ml/day, hemoptysis and fever. From his last sputum culture St.aureus was isolated. Lung function tests reveal a mixed ventilatory pattern (FVC 55%, IT 61%, FEV 40%), without impairment of diffusing lung capacity (TLCO 82,7%). Long term oxygen therapy, nebulized hypertonic saline solution, bronchodilators intermittent antibiotic administration (according to the bacteria’s sensibility) helps to prevent lung function decline and to improve his health-related quality of life. The particularity of this case is based on the severe, progressive pulmonary lesions since patient’s childhood, which etiology has not yet been elucidated, the choice of effective treatment being a major problem. Bronchiectasis is a heterogeneous disease. This case illustrates one of the most difficult task – identification of the etiology of bronchiectasis, that is an important point in the future management.