Combined pulmonary fibrosis and emphysema (CPFE) syndrome is a rare disease characterized with shortness of breath, upper lobe emphysema, lower lobe fibrosis and impairment of gas exchange. This syndrome is a disease usually seen in male smokers. Pulmonary hypertension is associated with mortality. Another important feature, spirometric volumes relatively protected and a reduction in carbon monoxide diffusion test. CPFE syndrome in the literature so far have been identified in only 70 patients. In this review CPFE syndrome is presented with literature.