Loeys-Dietz syndrome is a newly described entity characterised by a constellation of arterial tortuosity, cranial malformations, and hypertelorism. We report a case of a 7-year old boy with confirmed Loeys-Dietz syndrome and discuss magnetic resonance imaging as a complete technique for assessment and follow-up of aggressive vascular pathology in the brain, thorax, and abdomen, which may dictate early surgical intervention.