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Summary Abdominal aortic aneurysm (AAA) is an extremely rare condition in paediatric patients but can occur more frequently in patients with congenital connective tissue diseases such as Marfan syndrome or Loeys-Dietz syndrome (LDS). We report a case of an adolescent with known LDS presenting in the emergency department with spontaneous, acute lumbar back pain. Initially, the pain was managed as acute lumbago but later found to be caused by a ruptured AAA. Following urgent surgical intervention with a bifurcated prosthetic vascular graft, the patient recovered successfully. This case highlights the necessity of maintaining awareness of severe complications in LDS patients, even when alternative diagnoses appear more likely.