Takayasu's disease is a rare chronic vasculitis of unknown aetiology. Initial symptoms and signs are non-specific, and a high index of suspicion is needed to make the correct diagnosis. The disease is associated with a high incidence of morbidity, and a significant risk of premature death. Serological tests have proved unreliable in distinguishing active from quiescent disease, with non-invasive imaging currently offering the best option for early diagnosis, and monitoring the response to treatment. In this review, we detail the epidemiology, pathophysiology, clinical features, imaging characteristics, and currently available treatments.