Systemic sclerosis is a multisystem disorder of unknown etiology and involves organ systems such as the skin, peripheral vasculature, gastrointestinal tract, kidney, heart, and the lungs is observed quite frequently. In this review, we discuss the development of interstitial lung disease, one of the common pulmonary manifestations and a major cause of mortality and morbidity in this disorder. It is, however, under-recognized and diagnosed late in the course of the illness. Early use of pulmonary function tests followed by bronchoalveolar lavage in appropriate cases helps in early diagnosis. Recent studies emphasize the role of various profibrotic and inflammatory cytokines both locally in the lung and systemically in its pathogenesis. Treatment is helpful in arresting the progression if initiated early. Cyclophosphamide with or without corticosteroids given orally or as intravenous pulse may be helpful.