Objectives: Takayasu arteritis (TAK) is large-vessel vasculitis that predominantly affects young women. There is worldwide variation in the frequency of TAK depending on genetic and/or environmental factors. With Australian data lacking, we describe the epidemiology, clinical features and outcomes of TAK in Western Australia (WA). Methods: Retrospective case cohort study of incident TAK cases between 1 January 2000 and 30 June 2015 in WA identified from multiple sources. Data on disease presentation and outcome were extracted from medical records. Results: Eighteen patients received a clinical diagnosis with thirteen cases meeting 1990 ACR criteria for TAK (all female, 77% Caucasian, age at diagnosis 39 years) included in this analysis. Per million inhabitants the annual incidence and prevalence (2015) was 0.3 and 3.2 for Caucasians and 1.1 and 15 for Asians. Limb claudication and bruit were commonest symptoms; ESR or CRP was raised in 61% at diagnosis while aortic involvement Hata type IIb most prevalent (30%). Despite general combination therapy with corticosteroids and immunosuppressant drugs, four patients suffered a stroke, two patients developed myocardial infarction, and five patients required vascular intervention. Three Caucasian patients died during the study period. Conclusion: TAK is much more prevalent in the Asian than Caucasian population of WA and associates with significant long-term morbidity and mortality despite standard therapy.