Posterior cleft larynx is an uncommon congenital anomaly of variable extent representing an incomplete or absent separation of larynx and trachea from the hypopharynx and esophagus. This case report describes the histopathology of a type 2 cleft (classified according to Evans) associated with a subglottic stenosis due to elliptical shape of the cricoid cartilage and redundant submucosal tissue in a premature female infant.