Hypoplasia or absence of the epiglottis in an adult is a rarely reported congenital anomaly that usually occurs in association with congenital anomalies of other organ systems. Most epiglottic anomalies usually present in infancy and early childhood with respiratory and feeding problems and the affected individual dies shortly after birth due to multiple congenital anomalies. We present a case of congenital absence of the epiglottis and false vocal folds with hypoplastic maxillae in an adult.