Spontaneous rupture of a giant coronary artery aneurysm is a rare and almost uniformly fatal complication of Kawasaki disease. We report an 18-month-old boy with incomplete, immunoglobulin-resistant disease whose giant aneurysms ruptured despite pulse corticosteroids, anakinra, bedside sternotomy, and extracorporeal support. Six comparable paediatric cases are reviewed, underscoring that aggressive therapy may not prevent rupture once structural arterial wall destruction has occurred.