Epiglottis anomaly associated with Pierre Robin sequence (PRS) is a rare occurrence. Most infants with PRS have presented life-threatening symptoms of respiratory distress and severe feeding problems that usually end with death. To the knowledge of the authors, this is the first reported case of epiglottis agenesis associated with PRS. The clinician must be aware of this unusual presentation in a PRS, and the presented case reveals the challenges in the treatment of the respiratory and feeding problems. This case is discussed with a review of the literature. © 2010 Elsevier Ireland Ltd. All rights reserved.