An unusual case of hypoglossia-hypodactyly syndrome

Hypoglossia-hypodactyly syndrome is seen very rarely and its appearance is sporadic. Different degrees of tongue hypoplasia and transverse deficiencie s in the upper extremities are seen. In the patient presented there was a s ulcuslike deformity at the midline of the lower lip, and the continuity of the orbicularis oris muscle was disturbed at this location, in addition to the classic findings of hypoglossia- hypodactyly syndrome. A description of this variant and its treatment are described.