Background <p>Takayasu arteritis (TAK) is a rare granulomatous inflammatory vasculitis primarily affecting the aorta and its major branches. Data on childhood-onset TAK (c-TAK) remain scarce. This study retrospectively evaluates the clinical presentation, disease flares, treatment, and outcomes of c-TAK in a tertiary Canadian center.</p> Methods <p>We identified all children under 18 years of age at disease onset with a clinical diagnosis of TAK seen at Alberta Children’s Hospital, Calgary, Canada, between 2000 and 2024. Patients meeting the EULAR/PRINTO/Pres classification criteria for c-TAK were included. Baseline demographic data, clinical presentation, laboratory findings, imaging results, disease flares, and treatment were documented. Additionally, we highlight two challenging cases due to their particularly complex disease trajectories.</p> Results <p>Six children (4 female) with a median age at diagnosis of 14.5 years (range: 4–17) met the classification criteria for c-TAK. Clinical presentation was variable, with the most common symptoms being fatigue (<i>n</i> = 4), weight loss (<i>n</i> = 3), and hypertension (<i>n</i> = 3). The most frequently affected arteries were the abdominal aorta and carotid arteries (<i>n</i> = 5) followed by ascending aorta (<i>n</i> = 4). All patients received corticosteroids for induction treatment. Additional immunosuppressive therapies included methotrexate (<i>n</i> = 5), infliximab (<i>n</i> = 2), tocilizumab (<i>n</i> = 2), IVIG (<i>n</i> = 2), etanercept (<i>n</i> = 1), adalimumab (<i>n</i> = 1), and cyclophosphamide (<i>n</i> = 1).</p> Conclusions <p>TAK is a rare, potentially life-threatening large-vessel vasculitis. Early recognition is crucial for timely diagnosis and aggressive treatment initiation. Children with TAK often experience a complex disease course requiring multiple treatment adjustments and surgical or endovascular interventions. Large, multinational collaborations are essential for advancing our knowledge and improving patient outcomes.</p>