A syndrome associating an upper-lobe emphysema and pulmonary fibrosis of the lower lung was recently characterized. Few cases were identified in the literature. We report a case of a 68 year- old smoker man presented for exacerbation of a severe dyspnea. Physical examination revealed basal crackles and finger clubbing. Blood gas analysis showed hypoxemia. Chest radiography showed features compatible with emphysema of the upper lobes and fibrosis of the basis. Chest computed tomography confirmed chest radiography's findings and revealed fibrosis. The diagnosis of idiopathic pulmonary fibrosis was made. Pulmonary function tests showed obstructive pattern. Systolic pulmonary arterial pressure was elevated up to 87 mm Hg on heart ultrasonography. The authors emphasize the importance of the diagnosis of this entity made through chest computed tomography and the fact that it is characterized by severe impairment of gas exchange, the high prevalence of pulmonary hypertension and poor survival.