The Van der Woude syndrome is a rare autosomal-dominant craniofacial disorder. In its classical form consisting of a cleft lip or cleft palate, distinctive pits of the lower lips and hypodontia. This is a review of a family with VWS. The family pedigree, past history and clinical features were reviewed. The related literatures were studied, which show the variable presentations including lip pits alone, absent teeth, or isolated cleft lip and palate of varying degrees of severity, and the points for early diagnosis.