BACKGROUND: Systemic sclerosis (SSc), characterized by fibrosis of the skin and other organs, is a devastating systemic autoimmune disease. Lung involvement is frequent in SSc and contributes to the high rate of mortality. OBJECTIVE: To describe the first case report of SSc and interstitial lung disease (ILD) receiving autologous hematopoietic stem cell transplantation (ASCT) in Taiwan. METHODS: Case report. RESULTS: A 51-year-old man presented with rapid skin thickening and shortness of breath. Early progressive SSc-associated ILD was diagnosed. Because his lung function rapidly declined and his skin disease progressed, he received ASCT with satisfactory treatment responses in both skin thickness and lung function. In addition, lung imaging analysis showed remarkable improvements in ILD after treatment. CONCLUSIONS: We suggest that ASCT can be considered in selected patients with early, rapidly progressive SSc associated with ILD.