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Seventy cases of Takayasu arteritis in Korean children are reported. There were 57 females and 13 males (male-to-female ratio; 1:4.4). The youngest patient was a 3-year-old female. Family history was positive in one patient. The most common chief complaints on admission were dyspnea, headache, palpitation, and edema which were due to hypertension and congestive heart failure. Hypertension was seen in 65 out of 70 patients (92.8%). The abdominal aorta, thoracic aorta, and renal arteries were the most commonly involved sites in these children. Two patients had nephrotic syndrome. The frequency of positive tuberculin reaction was much higher in children with Takayasu arteritis compared with the general population, and the intensity of the reaction was also stronger. The majority of the patients required immediate medical treatment to control congestive heart failure due to hypertension at initial presentation. When ESR was elevated, corticosteroid was administered. Surgical treatment showed good results in six out of ten cases. Percutaneous intraluminal angioplasty was effective for lowering the blood pressure in six out of nine cases. In three cases, restenosis occurred and angioplasty was repeated in two cases.