The objective of the study is to report a case of atrial myxoma with bloodstream metastasis misdiagnosed to be Takayasu arteritis. A 23-year-old woman manifested with fever, repetitive vasocerebral events and extremities ischemic signs (claudication, difference of BP in arms and absence of pulse) for 5 years. Imaging studies revealed multiple cerebral infarction, bilateral iliac artery stenosis and as thrombosis formation. Takayasu arteritis was diagnosed according to American College of Rheumatology criteria. Glucosteroids and immunosuppressants were given; however, symptoms were not relieved even after treatment. Echocardiography suggested a mass in left atrium, which was surgically removed and pathologically confirmed to be atrial myxoma. Clinical symptoms completely relieved after operation. In conclusion, atrial myxoma with bloodstream metastasis sometimes presented with similar clinical symptoms of Takayasu arteritis and may lead to misdiagnosis of these two diseases. Usually, accepted diagnosis criteria are not enough in distinguishing these diseases. Echocardiography should be considered in these patients.