Purpose: Aglossia is a rare condition referring to the congenital absence of the entire tongue that is associated with activities such as sucking, swallowing, chewing, and phonation playing an important role in facial growth. The objective of this clinical case report is to demonstrate that adequate orthopedic and orthodontic procedures can re-adjust the buccal bone structures by redirecting the growth and the muscular adaptation, contributing to the improvement of the functions of the stomatognathic system. Methods: A girl 9 years and 4 mouths complained of difficulty to chew and diagnosed with congenital aglossia. The treatment included rapid maxillary expansion and mandibular midline distraction osteogenesis to correct transverse deficiencies and to provide conditions of myofunctional adaptation. It may occur in the course of growth and development of craniofacial complex. Results: Maxillary and Mandibular bones were successfully expanded allowing a better development of adaptive mechanisms to compensate for absence of the tongue. Conclusions: The use of a tooth-borne distraction device with a Hyrax-type screw to expand the mandibular by symphysis osteogenesis was efficient and improved the tongue tonus and occlusion. However, multidisciplinary continuous cares are required due to lack of proper stomatognathic function.