A 62-year-old Korean woman was admitted to our department to evaluate a chronic cough and sputum, which had begun several weeks ago. The patient had been diagnosed with systemic sclerosis in 2004. Autoantibody screening tests were negative for the anticentromere and antitopoisomerase antibodies. She received therapy with combined cyclophosphamide, a calcium channel blocker, D-penicillamine, and low dose steroid. In 2006, a pulmonary function test (PFT) showed a restrictive pattern, and a computed tomography (CT) scan of the lungs revealed interstitial lung disease, but no symptoms were present, so we maintained her on the medication. In October 2008, a chest x-ray and CT scan of the lungs demonstrated aggravation with bilateral basal interstitial infiltrates and hilar lymphadenopathy. Cyclophosphamide pulse therapy was conducted six times during 6 months, but there was no change on her chest CT and PFT, and she had no symptoms, so we decided to follow up. On admission, no significant interval change in the reticular opacity of both lower lungs was observed, but several lymph nodes were enlarged on a chest and neck CT. The skin showed multiple large polygonal-shaped scaled lesions on her upper and lower extremities. Biopsies were taken from the skin of the lower extremities and the left cervical lymph node. Typical non-caseating granulomas corresponding to sarcoidosis were found along with systemic sclerosis findings.