Pulmonary atresia with intact septum is itself a rare congenital abnormality, albeit known to be associated with other cardiac and non-cardiac anomalies. The combination of right- and left-sided obstructive lesions, however, is extremely rare. We describe a patient having pulmonary atresia with intact septum associated with aortic coarctation, which to the best of our knowledge has been previously described on but one occasion.