Chronic obstructive pulmonary disease (that includes emphysema) results in significant morbidity and mortality worldwide. Idiopathic pulmonary fibrosis (IPF) is also a chronic and progressive parenchymal lung disease with an average survival of less than 5 years after diagnosis. Combined pulmonary fibrosis and emphysema (CPFE) is an important but still underdiagnosed syndrome. Its diagnosis is based on the radiological findings at computed tomography which consists of emphysema of the upper lung zones and fibrosis of the lower lung zones. Since this syndrome has a very bad prognosis, even worse than isolated finding of emphysema or fibrosis alone, early recognition and rapid treatment are important. In this article we will review and elucidate the radiologic appearance of this syndrome and highlight its clinical importance.