Abstract: Objective To investigate the clinical features of antiphospholipid antibody-positive Takayasu arteritis and to enhance the awareness of its diagnosis and treatment. Methods The clinical features and imaging data of one patient with antiphospholipid antibody-positive Takayasu arteritis were retrospectively analyzed. Literature review was performed by searching the keywords “Takayasu arteritis” and “antibodies, antiphospholipid” or “antiphospholipid syndrome” in databases such as Wanfang, VIP, China National Knowledge Infrastructure （CNKI）, and PubMed. Results The 41 year-old male patient presented with intermittent abdominal pain and unconsciousness. The imaging findings showed multiple stenosis and occlusion of large arteries. The patient was combined with cardiovascular and cerebrovascular disease, renal failure and infection. Despite the treatment of anti-inflammatory drugs, anticoagulation, hormones, immunosuppressive agents, immunoglobulin, and plasma exchange, the clinical outcome did not improve. Through literature review, 8 cases of Takayasu arteritis with positive antiphospholipid antibodies, aged between 17 and 72 years were identified, including 2 males. Except for one death, the outcomes of the remaining 7 patients were improved through comprehensive treatment. Conclusion For patients with antiphospholipid antibody-positive Takayasu arteritis, early diagnosis, comprehensive evaluation, and stratified intervention are critical for successful diagnosis and treatment. Clinicians should enhance their awareness of early diagnosis and differential diagnosis, standardize anticoagulation and immunosuppressive therapy, and consider interventional and surgical treatment when necessary to improve the outcomes.