Introduction. Swyer-James-MacLeod syndrome (SJMS) or unilateral hyperlucent lung syndrome is a rare disorder caused by infectious bronchiolitis obliterans and pneumonitis occurring in childhood. It is characterized by hypoplasia and/or agenesis of the pulmonary arteries resulting in pulmonary parenchyma hypoperfusion. Materials and methods. We report the case of a 27 years-old female patient who presented with progressive dyspnea, productive cough, fever and chills. Results and discussion. Chest radiography showed unilateral loss of left lung volume with hyperlucency. Unilateral reduction in vascularity with reduced caliber of the left pulmonary artery was revealed on CT scan of the chest, final diagnosis of SJMS being confirmed by angiography. Conclusions. This case strongly supports the recommendation of considering SJMS within the differential diagnosis workup of bronchiectasis, the syndrome being usually underdiagnosed.