The Van der Woude syndrome is a rare autosomal dominant development malformation characterized by a paramedian lip pits and /or sinuses or conical elevation of lower lip associated with cleft lip and or palate. These congenital lip pits usually appear clinically in the vermilion border of lip, with or without secretion. The critical region of VWS has been identified to be at Iq32 to 41 with high, but incomplete penetrance and variable expressivity. Therapeutic intervention is generally required for cosmetic reason or when recurrent inflammation is present. Dental surgeon should be aware of this syndrome, as it is associated with variety of other congenital malformation. van der woude syndrome can be easily missed if it is not in the back of mind and its associated congenital malformation if present. We report a case of lower lip pits with bilateral cleft lip. Key words:Van der Woude Syndrome, congenital pits, cleft lip / palate.