Introduction: The prognosis and treatment of connective tissue disease related ILD (CTD-ILD) can be dissimilar from that of other forms of ILD, such as idiopathic pulmonary fibrosis (IPF). Therefore, lung transplantation for CTD-ILD remains contentious. This study was undertaken to evaluate the outcomes for CTD-ILD compared to IPF after lung transplantation. Method: We investigate a single-center retrospective study including lung transplantation recipients with CTD-ILD or IPF at tertiary hospital in South Korea between October 2012 and June 2016. Result: 19 patients with CTD-ILD underwent lung transplantation. Dermatomyositis/Polymyositis related ILD and Rheumatoid arthritis related ILD was the highest in 5(26.3%) patients each. Survival rate at 3 month, 6 month and 1 year post-transplantation was 100%, 83.3% and 81.8%. 1(9.1%) patient has increased activity of CTD after transplantation. Of the patients who survived more than 1 year after transplant, CTD-ILD (n=11) and age-and sex-matched IPF (n=11) was compared in a direct. The CTD-ILD group included fewer former smokers (37.3 vs. 72.7, p=0.033) compared with the IPF group. And pulmonary hypertension was more frequent in patients with CTD-ILD than in patients with IPF, although this difference was not statistically significant (62.5% vs. 42.9%, p=0.447). Over the following first year, there was no significant difference in survival between groups (81.8% vs. 100%, p=0.138). Conclusion: Patients with CTD-ILD who are recipients of lung transplantation experience similar survival rate compared with those with IPF.