Lacrimo-auriculo-dento-digital syndrome, which has an autosomal-dominant pattern of inheritance, typically affects the lacrimal apparatus, ears, teeth and hands. More recent case reports have uncovered involvement of the kidneys and the respiratory and skeletal systems. Salivary gland malformation may also be a feature of the syndrome, leading to dry mouth. The characteristics of the syndrome vary among individuals, but dental anomalies seem to be prevalent. Microdontia and hypodontia occur, possibly with gray enamel, wear, and caries. This case report introduces a new three-generation family of six individuals affected with lacrimo-auriculo-dento-digital syndrome and concentrates on the dental presentation and management of two of the members.