A 53-year-old woman with Takayasu arteritis was admitted to hospital because of worsening exertional angina. Coronary angiography revealed 90% ostial stenosis in the left main coronary artery (LMCA), which also involved the bifurcation of the relatively short LMCA. Because the patient refused coronary bypass surgery, she underwent percutaneous coronary intervention (PCI) and the stenosis was successfully dilated. However, the exertional angina recurred a few months later and again after the second PCI. Finally, a sirolimus-eluting stent was deployed in the in-stent restenotic lesion. The patient has been free from angina pectoris for 6 months after the last PCI and follow-up coronary angiography indicated no restenosis in the LMCA.