Congenital lower lip pits are a rare developmental malformation of the lower lip. Clinically they present as bilateral depressions in the vermilion zone of the lower lip. It is important to be aware of this disorder because lower lip pits have also been reported in a variety of other congenital disorders and are associated with other malformations. Ten cases of this entity are presented. Nine of the 10 patients are members of two related families. The clinical and pathologic picture as well as some of the genetic and therapeutic aspects of this peculiar condition are discussed.