ABS TRACT Dental hypodontia (agenesis) is a common congenital anomaly. One or more missing teeth are more common, but multiple missing teeth are rare. In oligodontia, tooth defect is not usually seen as a segment involvement. The segmental involvement is the first regional odontodisplasia (RO) in mind. However, hand-foot and tongue problems are not characteristic in RO. This suggests that we have a syndrome in our case. In this case report, it is aimed to present and discuss the patient who has sebaceous, palpable adhesions in the hands and feet and missing primary and permanent teeth in a segment of the lower jaw. In this case, which is also likely to be a syndrome, our colleagues were also asked to get ideas.