Objective Few studies have evaluated survival of Takayasu's arteritis (TAK; M31.4) in Korea. The purpose of this study was to assess the survival rate (SR) and causes of death for TAK. Methods Newly diagnosed TAK data (N = 2731) were collected from the National Health Insurance Service in Korea from 2006 through 2017. The Kaplan-Meier method was used. Korean death data was used from 2006 through 2018. Results The mean age was 48.1 (+/- 16.9) years. The proportion of female patients was 74.4%. The most common cause of death in TAK was diseases of the circulatory system. The 1-, 3-, 5-, and 10-year SRs were 97.5%, 94.7%, 91.7%, and 84.7%, respectively. The 1-, 3-, 5-, and 10-year SRs by gender were 97.8%, 95.6%, 92.9%, and 86.3%, respectively, among females and 96.8%, 92.2%, 88.4%s and 79.7%, respectively, among males (P < .001). Conclusions The overall 10-year SR was about 85%. The 10-year SR in males was lower than that in females. The most common cause of death in TAK was diseases of the circulatory system.