A 43-year-old white man and his 12-year-old daughter both displayed congenital lower lip pits. Since Van der Woude's description of this syndrome more than forty years ago, little attention has been given to this uncommon anomaly, probably due to variable expressivity, incomplete penetrance, and recognition of its clinical stigmata. We describe two family members with Van der Woude's syndrome and review its clinical aspects, differential diagnosis, and treatment.