Takayasu arteritis is an idiopathic chronic granulomatous panarteritis predominantly affecting the aorta and its main branches. Although idiopathic, genetic contribution to disease susceptibility is being increasingly recognised. Rare in children, Takayasu arteritis is a worldwide disease with significant morbidity and mortality. Its diagnosis is a challenge and requires awareness of the condition as clinical features at presentation are non-specific and assessing disease activity is difficult. In the inflammatory stage, treatment is essential to prevent the insidious course and vascular damage: stenotic, occlusive lesions, aneurysms, and aortic regurgitation. New imaging modalities, such as CT scan, MRI, and 18F-fluorodeoxyglucose positron emission tomography, have expanded the possibilities for non-invasive diagnosis and monitoring; however, digital subtraction arteriography remains the gold standard for the diagnosis of Takayasu arteritis. Steroids are the first-line medical treatment. The combined use of methotrexate, cyclophosphamide, azathioprine, mycophenolate mofetil, and biological agents is common. Revascularisation therapy should be considered in uncontrolled hypertension secondary to renal artery stenosis, symptomatic coronary ischaemia, cerebrovascular disease, severe aortic regurgitation, limb ischaemia, and aneurysms at risk of rupture, using surgical or endovascular procedures and taking into consideration that complications, especially restenosis, are frequent. Disease activity increases the likelihood of complications after revascularisation. Surgical intervention has shown better long-term outcomes, although the endovascular approach is evolving. The aim of this review was to describe key points of the diagnosis, treatment, and follow-up of Takayasu arteritis in childhood.