The epiglottis plays an important role during swallowing to prevent food penetrating into the airway by closing down the laryngeal vestibule during laryngeal elevation. In case of the absence of the epiglottis, airway closure might fail. The absence of the epiglottis is rarely seen in patients with Pierre Robin Sequence (PRS). We report here the swallowing problems of a PRS case with absence of the epiglottis and the results of our interventions. A male infant who was born by cesarean section with a weight of 3 kilograms was diagnosed with PRS. When he was 11 months old, he was referred to Hacettepe University, Department of Physiotherapy and Rehabilitation, Swallowing Disorders Units due to a history of aspiration pneumonia. First, a fiberoptic endoscopic swallowing evaluation (FEES) was performed and the absence of the epiglottis was recognized. Because of the uncomfortable feeling during FEES, videofluoroscopic swallowing evaluation (VFSE) was also performed for a more detailed swallowing evaluation. Aspiration was seen. A nasogastric tube was inserted after VFSE, and a swallowing therapy program was followed for 2 months.Thermal tactile stimulation to trigger swallowing reflex, laryngeal mobilization to support hyolaryngeal elevation, and neuromuscular electrical stimulation were used. VFSE was repeated after rehabilitation. Aspiration of liquid consistency continued, but he tolerated pudding consistency. At that time, liquid-restricted oral intake was started. Early diagnosis and intervention is very important in patients with absence of the epiglottis.