Bifid epiglottis is a rare congenital defect that is often associated with other congenital annomalies. The most common defect associated with a bifid epiglottis are anomalies of the hands and/or feet (90%) while the most dangerous and potentially lethal anomaly if not recognized and treated are hypothalamic hamartomas and hypopituitarism (50%). A bifid epiglottis will often result in severe respiratory distress secondary to laxity of the cartilage and chronic aspirations, which may require surgical intervention. We present the case of a 10-week-old child who was sent for evaluation of stridor and aspiration. Office laryngoscopy demonstrated a true bifid epiglottis and further evaluation demonstrated a hypothalamic hamartoma consistent with Pallister-Hall syndrome. Management of our case as well as those previously presented in the literature are reviewed.
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