Congenital bronchial atresia is rarely symptomatic in adults. Recurrent lung infection and pneumothorax are the feared complications of this otherwise benign condition. The objective of this article is to present a case of congenital bronchial atresia manifesting as pulmonary hypertension in a 66 year-old patient. While doing so, we highlight the relevant knowledge accrued in medical literature with regards this rare condition. Finally, with the revelation that congenital bronchial atresia may cause severe pulmonary hypertension later in life, perhaps a less conservative approach to management may be warranted in younger adults and children with this condition.