Scleroderma is an autoimmune connective tissue disorder which is characterized by fibrosis of visceral organs, blood vessels and skin. The most common manifestations of lung disease in systemic sclerosis are interstitial lung disease and pulmonary hypertension and, together, are the leading cause of mortality in systemic sclerosis. Recently, we notice a new pattern called Combined-pulmonary emphysema and lung fibrosis. Most patients with this entity are male smokers or ex-smokers. This entity is characterized by the coexistence of both centro-lobular and para-septal emphysema in the upper lobes and interstitial lung disease in the lower lobes. Here, we present a case of a nonsmoker adult woman with systemic sclerosis, in which High Resolution Computed Tomography of lung showed combined fibrosis and emphysema with atypical radiological presentation and unusual distribution. This case outlines the importance of recognizing the presence of combined fibrosis and emphysema in patient with systemic sclerosis even without smoking history.