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The long-term outcomes and optimal treatment strategy for patients with Takayasu arteritis (TA) and significant coronary stenosis remain unclear. We aim to investigate the prognosis of these patients according to the initial management strategy. A total of 57 consecutive patients with TA and significant coronary stenosis were included between 2002 and 2018. The cohort was divided into the percutaneous coronary intervention (PCI) group (n = 18), coronary artery bypass graft (CABG) group (n = 10), and medical-therapy group (n = 29). The primary outcome was major adverse cardiac events (MACEs) defined as a composite of cardiac death, myocardial infarction, and coronary revascularization. Over a median follow-up of 4.5 (IQR 3.5–8.0) years, 24 (42.1%) patients experienced at least one MACE. The long-term rates of MACEs and re-revascularization were significantly higher in the PCI group than in the other 2 groups (HR 5.306, 95% CI 2.160–13.036, p < 0.001; HR 12.286, 95% CI 3.257–46.343, p < 0.001, respectively). The cumulative incidences of MACEs and subsequent revascularization were similar between the CABG and medical-therapy group. Active disease at baseline and PCI were independent predictors of MACEs (HR, 7.039, 95% CI 2.031–24.396; p = 0.002; HR, 4.400; 95% CI 1.804–10.727; p = 0.001, respectively) and revascularization (HR 4.632, 95% CI 1.010–21.235, p = 0.048; HR 9.820, 95% CI 2.641–36.514, p = 0.001, respectively). The initial baseline active disease is an important predictor of long-term outcome and subsequent revascularization in patients with TA and significant coronary artery stenosis. Also, PCI is independently associated with higher risk of MACEs and re-revascularization. • Active disease at baseline was an independent predictor of MACE in patients with TA and significant coronary stenosis. • Compared to CABG and medical therapy group, PCI group was independently associated with an increased risk of MACE. • An initial strategy of medical therapy showed comparable long-term outcomes to initial CABG in TA patients. [ABSTRACT FROM AUTHOR]