BACKGROUND Takayasu arteritis (TA) is a chronic inflammatory disease involving the aorta and its major branches. Initial diagnosis is usually difficult due to the highly variable symptoms. Acute myocardial infarction (AMI) is a very rare presentation in patients with TA. Moreover, the choice of early management for these patients is not well established. METHODS A 34-year-old woman was taken to the Emergency Department of our hospital, presenting with a sudden onset and persistent retrosternal chest pain radiating to both upper extremities for 2 hours. Blood pressures were different between 2 arms with 151/115 mm Hg on the right arm and 140/100 mm Hg on the left arm. METHODS The patient was diagnosed with TA according to the medical history, physical examination, and vascular imaging. METHODS Primary percutaneous coronary intervention (PPCI) was performed to restore the coronary flow of left anterior descending. Meanwhile, combination of oral glucocorticoids and immunosuppressive agents was administered to halt disease progression of TA. RESULTS Chest pain was relieved without rest and exertional angina. The patient achieved long-term remission without symptom relapse during our follow-up. CONCLUSIONS Percutaneous coronary intervention was essential and effective in AMI of TA. Timely immunosuppressive therapy could improve the long-term outcome.