Takayasu's arteritis (TAK) is a rare large vessel vasculitis of unknown etiology that chiefly targets the aorta and its branches. It predominantly affects females under 50 years of age. A relationship between TAK and Mycobacterium tuberculosis (TB) has been suggested for a long time, but only a few systematic studies have been done centering on this association. The present systematic review aimed to analyze the possible association between TAK and TB based on the studies conducted previously. A detailed search was conducted until April 2021 using three databases: PubMed, Cochrane Library, and MedlinePlus. PubMed search on the related topic identified 1053 articles, four on Cochrane Library, and three on MedlinePlus. Finally, 13 papers were pertinent for our review. The appropriate data was extracted from these articles, and the risk of bias assessment was done. The systematic review of these finalized articles found that the majority of the current studies supported the presence of TB in patients with TAK. Out of 13 final observational studies, only one study failed to detect a link between TAK and TB. However, data are still lacking that show a direct link between them. Future large-scale studies are needed to probe the exact role of Mycobacterium tuberculosis infection in the etiopathogenesis of TAK.