In this case report, we present the orthodontic and surgical management of an 18-year-old girl who had a severe craniofacial deformity, including maxillary prognathism, vertical maxillary excess (gummy smile), mandibular retrognathism, receding chin, and facial asymmetry caused by unilateral temporomandibular joint ankylosis. For correction of the facial asymmetry, the patient's right mandibular ramus and body were lengthened via distraction osteogenesis after 5 months of preoperative orthodontic therapy. Subsequently, extraction of 4 first premolars, bimaxillary anterior segmental osteotomy, and genioplasty were simultaneously performed in the second-stage operation to correct the skeletal deformities in the sagittal and vertical planes. Postoperative orthodontic treatment completed the final occlusal adjustment. The total active treatment period lasted approximately 30 months. The clinical results show that the patient's facial esthetics were significantly improved with minimal surgical invasion and distress, and a desirable occlusion was achieved. These pleasing results were maintained during the 5-year follow-up.