Background: Congenital short trachea (CST) an uncommon abnormality is defined as a reduction in the number of tracheal rings to 15 or less, compared to the average of 17 rings in normal infants. As a result, the carina is situated at a higher level than usual that lead the left main bronchus to course abnormally behind the arch of the aorta, making it prone to compression. CST is associated with a number of syndromes, including the Di George syndrome, several types of congenital heart disease, some skeletal dysplasias and meningomyelocoele. Case study: We report a case of one Saudi infant who presented with respiratory distress since birth and hyperinflation of left side of the lung secondary to extrinsic narrowing of proximal left main stem bronchus due to compression by arch of the aorta, and he underwent successful aortopexy of the aortic arch for this condition.