Background: Distal aortic occlusion in pediatric patients with Takayasu arteritis (TA) has been very challenging to treat, especially in choosing a definitive revascularization strategy. Stenting significantly increases patency; however, the diameter might be undersized when the patient reaches adulthood. Case Summary: We reported a case of a 13-year-old girl with distal aortic occlusion caused by TA, which was successfully treated with drug-coated balloons (DCBs) in which the patency can be maintained over 2 years. Discussion: DCBs, mainly paclitaxel, help mitigate the risk of restenosis by inhibiting the proliferation of smooth muscle and intimal hyperplasia, which are both key factors of restenosis in inflammatory vascular conditions such as TA. Take-Home Message: DCBs might be a promising alternative to treat pediatric patients with TA presenting with distal aortic occlusion as it could help maintain patency while still giving chances for the arteries to grow until the patient reaches adulthood. © 2026 The Authors