Combined pulmonary fibrosis and emphysema syndrome (CPFE) is a newly defined syndrome with unique clinical findings, characterized by presence of emphysema radiologically. Since awareness of the syndrome is insufficient, CPFE is under-recognized. Male patient presented with progressive dyspnea and history of smoking 60 packs per year. Upper lobe emphysema and lower lobe fibrosis, which are specific for CPFE syndrome, were observed in chest computed tomography scans. Forced expiratory volume 1 was relatively well preserved, but diffusing capacity of the lungs for carbon monoxide was very low, and is an important feature of the disease. Pulmonary hypertension has also been determined to be characteristic finding of the syndrome. Patient was diagnosed as CPFE syndrome based on presence of all characteristic features and case is presented with review of the literature to draw attention to this disease.