Congenital hypothyroidism presents with characteristic symptoms within the first few months of life: constipation, jaundice, poor feeding, hoarse cry, macroglossia, and hypotonia. However, these symptoms are rare immediately upon delivery, as most neonates are asymptomatic at birth. Therefore, the newborn screens are essential in detecting congenital hypothyroidism, but there are rare cases in which congenital hypothyroidism can be missed or present before the first newborn screen. This paper presents a case of respiratory distress syndrome in a term neonate as the presenting symptom of congenital hypothyroidism. Overall, clinicians should be aware that neonatal respiratory distress can indeed be caused by congenital hypothyroidism, though more common etiologies should be ruled out following stabilization of the patient. Congenital hypothyroidism is an endocrinopathy that may present prior to the onset of symptoms at 3 months or may even present as respiratory distress syndrome prior to the results of the newborn screens. A discussion of this rare case may help physicians in the recognition and treatment of respiratory distress syndrome due to hypothyroidism.