Emphysema and idiopathic pulmonary fibrosis (IPF), with great differences in radiological, pathophysiological, therapeutic and prognostic characteristics, are regarded as 2 separate entities. However, some patients may present both the radiological features of emphysema and fibrosis at the same time, and it is named as combined pulmonary fibrosis and emphysema (CPFE). Being different from emphysema and IPF, CPFE has been recognized as an independent disease, and draws more and more attentions. We reviewed the causes and pathogenesis, clinical manifestations, diagnosis, complications, therapy and prognosis of CPFE in this article.