Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by fibrosis of the skin and internal organs, which can cause significant morbidity and mortality. The prognostic factors for survival were not fully evaluated in Asian population. We investigated the prognostic factors for survival of SSc among Korean patients. A total of 243 SSc patients were enrolled from Seoul National University Hospital between 1972 and 2007. Age at onset, gender, cutaneous subset, autoantibody status, major organ involvement, and occurrence of malignancy were evaluated with all-cause mortality as the end point. A multivariate Cox proportional hazard model was used to retrieve the prognostic factors for survival. During the follow-up of 1,967 person-years, 33 patients died. Old age at onset (hazard ratio [HR] 7.4, 95% confidence interval [95% CI] 1.9-28.1), diffuse cutaneous subset (HR 2.5, 95% CI 1.1-5.9), presence of anti-Scl-70 antibody (HR 3.0, 95% CI 1.2-7.1), forced vital capacity less than 70% (HR 2.8, 95% CI 1.3-6.2), and heart involvement (HR 4.2, 95% CI 1.7-10.2) were found to be significant risk factors for mortality in multivariate analysis. In Korean SSc patients, old age, diffuse cutaneous involvement, anti-Scl-70 antibody, and internal organ involvement are risk factors for mortality.