We report the occurrence of a pyriform sinus cyst in a patient with Ellis-van Crevald syndrome. The patient presented with congenital stridor. The cyst was drained but recurred. MRI scanning revealed extensive involvement in the neck. Marsupialization and CO2 laser ablation of the lesion was successful, and the patient remained free of symptoms. Although oral lesions have been described in association with this syndrome, to our knowledge this is the first case report of an upper airway cyst in a patient with the Ellis-van Crevald syndrome.