Van der Woude syndrome is an autosomal dominant disease characterized by lower lip pits with or without cleft lip and/or cleft palate. The lip pits commonly have salivary glands that drain into them, which leads to salivary flow from the lip pits. Lip pits may be associated with submucosal palatal cleft, velopharyngeal insufficiency, or genitourinary or cardiovascular anomalies. The pits are treated by surgical resection. The authors report a case of van der Woude syndrome with isolated lip pits and speech difficulties that had been unrecognized until the patient was 6 years old. The surgical technique is described to ensure that the often-bifurcating tracts are removed entirely.