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Van der Woude syndrome is a rare developmental disorder characterized by a combination of congenital lower lip pits, cleft palate alone or with cleft lip in some cases, and hypodontia. It occurs in approximately 2% of the population with facial clefts. Its prevalence varies from one in 100,000 to 200,000 births. This case report details a unique case of Vander Woude syndrome in a seven-year-old girl and her mother, with median lip pits, cleft lip and cleft palate, missing lateral incisors, and retained deciduous teeth, along with transposition of teeth.