Nager syndrome, or preaxial acrofacial dysostosis, is associated with aberrant development of the first and second branchial arch structures, as well as abnormal development of the limb buds. It is a rare disorder, and its clinical manifestations have not been completely defined. Absence of the soft palate has been reported in patients with Nager syndrome. In this report we describe the use of an elongated, superiorly based pharyngeal flap for the treatment of severe velopharyngeal insufficiency in a patient with Nager syndrome and absence of the soft palate. We also describe the dysmorphisms associated with Nager syndrome and present a differential diagnosis for the condition.