Choanal atresia is a relatively uncommon condition with an estimated incidence of 1:7000 live births. In 1755, Roederer was the first to describe it as an entity.1 In the following decade, further understanding of the subcategories of this deformity and its relationship to the palatine bones was developed.2 There are several theories that discuss the embryological development of choanal atresia. The purpose of this report is to highlight a clinical observation noted during the assessment and treatment of a patient with choanal atresia that highlights one of the embryological theories and a rare genetic correlation.