Waardenburg Syndrome(WAS) is a rare multiple congenital anomaly syndrome. This syndrome is characterized by hete-rochromia iris, dystopia canthorum, white forelock, depigmentation of skin and congenital hearing impairment. This article presents a case of 3-year-old boy with Waardenburg Syndrome(WAS). Because of his multiple caries, hearing loss and uncooperative behavior, we treated his caries under general anesthesia. The clinical manifestations, mode of inheritance, diagnostic criteria and clinical classification of Waardenburg syndrome are also addressed.