In 1962, a case of "generalized skeletal dysplasia with multiple anomalies" was reported by Taybi as a new clinical syndrome. The most characteristic features of the syndrome seemed to be hearing loss, cleft palate and peculiar digital anomalies, so, the syndrome designated as otopalatodigital syndrome by Dudding, et al. Recently, The authors have experienced a case OPD syndrome who visited Yeungnam University Hospital with the chief complaint of hypernasality, and underwent pharyngoplasty for correction of VPI. We present here a new case and review the literature on the subject.