Van der Woude syndrome (VWS) is a rare craniofacial anomaly with autosomal dominant inheritance and remarkable heterogeneity in expression. It manifests as lower lip pits with or without a cleft lip and/or cleft palate. The critical importance of lip pits is that their occurrence may be associated with a phenotypic severity of the cleft lip and cleft palate. The clinical manifestation of lower lip pits covers a wide spectrum. These include slight depressions on the vermilion border of the lip, and fistulas that penetrate into subjacent salivary glands and drain small amounts of saliva. Lip pits are usually circular or oval shaped, but have also been described as transverse, slit-like, or sulci. They may vary from an asymptomatic slight depression on the vermilion border of the lower lip to deep fistulas. The relatively high incidence in females has been attributed to the established fact that they are more likely to seek surgical intervention for cosmetic considerations. Cosmetic considerations are thus the most common indication for surgical intervention; however, some patients with asymptomatic lesions or mild symptoms may neither require nor request surgery. The goals of treatment are two-fold; removal of sinuses and providing cosmetic relieve for the disfigurement. The identification of familial lip pits is crucial for genetic counseling. Physical examination of relatives and interviews with older relatives are considered essential to identify affected family members. Information regarding the pattern of inheritance and the consequence of these phenotypes must be emphasized. Here we report an interesting case of VWS seen at the Ahmadu Bello University Teaching Hospital, Zaria, Nigeria, to highlight its peculiar clinical presentation and management.