<p>Many lung diseases are smoking-related. Among them, tumors, centrilobular and bullous paraseptal emphysema and fibrosing interstitial lung diseases (fibrosing ILDs), especially those with a usual interstitial pneumonia (UIP) pattern, are the most important entities. One may think it is easy to recognize emphysema on a chest X-ray (CXR) or on a high-resolution computed tomography (HRCT). However, the combination of emphysema and fibrosis can be found in many interstitial lung diseases, characterized by different radiologic features, course and prognosis. Moreover, quite frequently more than just one pattern can be detected in a single patient on biopsy, lung function tests and imaging. This may lead to diagnostic challenges and consequent misdiagnosis, as it all depends on the radiologist’s knowledge and experience. However, these entities are well known and the purpose of this article is to describe the less common radiologic features of the association of emphysema and fibrosis. In fact, we will discuss the case of a patient affected by airspace enlargement with fibrosis (AEF), which is thought to have a stable course, without exacerbations and progressive worsening of symptoms, lung function tests and imaging. However, our patient, whose lung samples also underwent pathologic evaluation, is an exception.</p>