We report a case of an 11-year-old girl presenting with Apert syndrome characterized by midface concavity, protrusion of the eyeballs, and ocular hypertelorism. She had class III anterior crossbite, narrow upper and lower arches, and marked crowding. Based on cephalometric analysis, anterior crossbite associated with marked midfacial hypoplasia was diagnosed. Orthodontic treatment in combination with Le Fort II maxillary distraction was scheduled. The dentition was laterally extended using a Rapid palatal expander in the upper jaw and a Bihelix in the lower jaw. Multi-bracket appliances were simultaneously applied for leveling. Next, Le Fort II maxillary osteotomy was performed to distract the midface bone 16 mm anteroinferiorly using a rigid external distraction system. Orthodontic treatment was completed at 3.8 years after initiation. Bone distraction moved the upper jaw anteriorly downward, and the lower jaw subsequently rotated posteriorly downward, leading to a marked improvement in facial appearance and occlusion. Elongation of the dorsum of the nose, in particular, allowed esthetic improvement of the saddle nose. These improvements remain stable at 2 years after orthodontic treatment.