RESUMO
Hemifacial microsomia (HFM) is a complex congenital malformation with an extremely variable phenotypic presentation. It usually involves structures of the first and second pharyngeal arches. Anomalies of the cardiac, pulmonary, renal, and gastrointestinal systems are present, but the main characteristic is the mandibular hypoplasia. This is commonly treated with orthodontic hardware and various surgical modalities. Most recently, a total joint replacement with a customized prosthesis is idealized to provide the best outcomes to these patients, so it has been used in some cases. The following case is of a 23-year-old female with congenital hypoplastic mandibular head and the absence of mandibular fossa. The proposed treatment was to reconstruct the mandible with a customized prosthesis and orthognathic surgery to correct the asymmetry and provide better phonation, speech, and facial contour. The patient is under six years follow-up with a complete adaptation of the prosthesis.