We reviewed the clinical features of 26 children with Takayasu's arteritis. In contrast to what is often seen in adults, in this age group inflammation of different systems is a conspicuous feature. Eighty five percent of cases had arterial hypertension. The high incidence of arthritis and heart failure provoked confusion with rheumatic fever. There was a high frequency of positive purified protein derivative (73%) and lymphadenopathy (38%) with a histological picture of caseating granulomas, suggesting a link between Takayasu's arteritis and an atypical mycobacterial infection. No improvement of the arterial obstruction was observed with corticosteroid therapy. Takayasu's arteritis in children presents as an aggressive often lethal disease. The mortality rate was 35% over an observation period of 5 years.