Witkop′s tooth and nail syndrome is a rare autosomal dominant disorder of ectodermal dysplasia characterized by hypodontia and nail dysplasia. Mutations in MSX-1 have been shown to be associated with this syndrome. There is failure of development and eruption of the dentition. Tooth shape may vary; the most common forms are conical and narrow crowns. The nails may be spoon shaped and slow growing and affect both finger and toe nails. The nail involvement is more severe in childhood. The present case describes a 14-year-old boy who showed the characteristic features of Witkop′s syndrome. A multifaceted approach to the dental management of the patient is discussed.