Takayasu's arteritis (TA) often referred to as pulseless disease, is a chronic inflammatory disorder affecting the aorta and its main branches. While the first reported case was documented in Japan in 1908, it does occur worldwide, but is more prevalent in young oriental females from China and Southeast Asia. The main complications of the disease are due to occlusion of major branches of the aorta. During the initial stages, one of the presenting symptoms may be jaw pain. This may prove to be a diagnostic challenge for the dentist, since many diseases cause orofacial pain and the diagnosis must be established before final treatment. A literature review of TA is presented along with clinical presentation and treatment modalities. A case report is also presented.