GCA is a common primary systemic vasculitis that results in granulomatous inflammation of medium to large arteries. Both innate and adaptive immune mechanisms combine to drive intimal hyperplasia, luminal stenosis and ultimately occlusion. While the pathogenesis of GCA is incompletely understood, the activation of resident adventitial dendritic cells via toll like receptors (TLRs) appears to be a crucial inciting event. Here we explore the role of TLRs in the pathogenesis of GCA, including their effects on dendritic cell and T cell activation and recruitment, putative infectious triggers for GCA and the potential of TLR inhibition as a novel therapeutic strategy in GCA.