Pulmonary artery sling is an uncommon entity in the neonatal period. It is defined as an abnormally originated left pulmonary artery arising from the posterior aspect of right pulmonary artery. Because of the associated structures in this region, mostly originating from sixth aortic arch, tracheobronchial anomalies and congenital heart defects, it frequently accompanies the pulmonary artery sling. Etiology of pulmonary artery sling has not been determined to date. Surgical correction is necessary even for asymptomatic cases because of the high mortality. Postoperative mortality is commonly associated with airway defects. Therefore, prompt diagnosis and timely intervention decrease the morbidity and mortality. We, herein, present a case of a neonate with isolated pulmonary artery sling un-associated with their developmental anomalies. Antenatal history was positive for maternal hypothyroidism, for which she was taking L-thyroxine. To the best of our knowledge, this is the first pulmonary artery sling case accompanied by maternal hypothyroidism. Key Words: Pulmonary artery sling, Neonate, Hypothyroidism.

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