A case of craniofacial duplication is presented. Details on this rare form are described, and its treatment is discussed with brief review of the pertinent literature. We excised the duplicate maxilla and also discovered bilateral macrostomia. A year later, a mass appeared again. By CT scan, we found that there was a mass in the skull base extruding to the superior wall of cavitas pharyngis. The mass below the sphenoid bone and the ethmoid bone connected with the skull base. CT scan also showed malformation of the first cervical vertebra and odontoid process had bifurcated. Once again, we excised the mass and found a cranial meningocele on the skull base, repaired the palate cleft, and closed the cerebral meningocele. The patient had a palate fistula after operation. A year later, the palate fistula and macrostomia were repaired. We think the patient should be operated on immediately after she was born so that we could relieve the dyspnea; furthermore, by one well-planned operation, we could repair the palate cleft and other deformity just after we excised the mass.