Keutel Syndrome is a rare autosomal recessive syndrome. Laryngotracheobronchial calcification is a rare finding in young children and can occur in Keutel syndrome. Here we report 6 years old female patient with laryngotracheobronchial and aortic calcification and also review and discuss the clinical features of the syndrome. Concentric calcification of hepatic, renal, coronary and cerebral arteries were mentioned in Keutel Syndrome but to the best of our knowledge no previous aortic calcification was reported.