Abstract: Witkop tooth and nail syndrome is a rare autosomal dominant ectodermal dysplasia manifested by hypodontia and nail dysplasia. Tooth shape may vary, and conical and narrow crowns are common. Mandibular incisors, second molars, and maxillary canines are the most frequently missing teeth. Nail dysplasia affects the fingernails and especially the toenails and is often more severe in childhood. Nails may be spoon-shaped, ridged, slow-growing, and easily broken. We examined an Eastern Mediterranean family in which three siblings (two girls, one boy) had this syndrome. The severity of finger and toenail anomalies varied. The children’s mother had no Witkop-related abnormalities, but their father was reported to have dental and nail anomalies. Early intervention is recommended to manage orofacial disfigurement and avoid negative social consequences for these children.