Bifid epiglottis associated with Joubert's syndrome

This report presents the case of a 2-year-old boy who had a bifid epiglotti s associated with Joubert's syndrome. He had episodic tachypnea and apnea, inspiratory strider, aspiration, and growth and mental retardation. Direct laryngoscopy demonstrated agenesis of the right half of the epiglottis and hypertrophied mucosa over the arytenoid cartilage. After the hypertrophied mucosa was partially vaporized with a CO2 laser, the inspiratory strider so on improved and the aspiration was alleviated. Brain magnetic resonance ima ging showed cerebellar vermian agenesis and enlargement of the fourth ventr icle.