Abstract： Objective To retrospectively analyze clinical presentations, pulmonary function parameters and radiological appearance in patients with combined pulmonary fibrosis and emphysema (CPFE) syndrome. Methods We reviewed the clinical features, imaging, and lung function indices from 8 patients diagnosed with CPFE according to the findings by high resolution computerized tomography (HRCT) since 2006 to 2009 at Peking Union Medical College Hospital. Results All patients were male, aged 65 (59-75) years, and 7 of them were smokers. Dyspnea on exertion was presented in 7 patients. Basal crackles were heard in 6 patients and finger clubbing was observed in 4 patients. Pulmonary function demonstrated that forced expiratory volume in one second/forced vital capacity (FEV1/FVC) was slightly lower with a median of 76% (range 60% to 86% ) , forced vital capacity (FVC) was 73% (51% -92%), and total lung capacity (TLC) was 80% (59% - 114%). However, carbon monoxide diffusion capacity (DLCO) was significantly impaired (44% , 16% -65% ). HRCT findings included emphysema predominantly at the upper zone, while reticular opacities, honeycombing and traction bronchiectasis were in the lower lobes. Pulmonary hypertension was seen in 6 patients. Conclusions The co-existence of lower lung fibrosis and upper lung emphysema was found in some smokers. Patients with CPFE syndrome can present with a normal or nearly normal lung volume but a remarkable impairment in gas exchange. A high prevalence of pulmonary hypertension is seen in CPFE syndrome. Further studies to elucidate the pathogenesis and to explore the treatment and prognosis are warranted.