Desquamative interstitial pneumonia is a type of smoking-associated major idiopathic interstitial pneumonia, which is characterized by accumulation of alveolar macrophages in alveolar lumens and septa and develops secondary to mainly active or passive exposure to cigarette smoke. Desquamative interstitial pneumonia mostly occurs in male smokers in association with non-specific symptoms responsive to steroid therapy and has a better prognosis than usual interstitial pneumonia. To date, no large-scale clinical studies have been performed on desquamative interstitial pneumonia patients. Factors responsible for the scarcity of data on the clinical course of this condition include the retrospective nature of the available information as well as its rare occurrence. Despite this, a general consensus exists as to the nature of its symptoms, association with smoking, age and gender distribution, findings of respiratory function tests, steroid responsivity and mortality. The objective of the present review article was to report on desquamative interstitial pneumonia and to describe its etiology, risk factors and clinical features, as well as the laboratory, bronchoalveolar lavage, radiological and histopathological findings, and the treatment and prognosis of affected patients.