Lung involvement in systemic sclerosis(SSC) is common but usually occurs late in the course. Skin changes usually occur before the pulmonary findings. In this report, a patient who developed pulmonary interstitial fibrosis without skin changes is presented. A diagnosis of SSC lung involvement was made histologically. The a nti-scl-70 antibody test was positive. Esophageal manometry revealed a lower amplitude in the lower two-third of the esophagus and pressure in the lower esophageal sphincter. Here we report a case of systemic sclerosis sine scleroderma presenting as pulmonary interstitial fibrosis with a review of the relevant literatures.