Cervical spinal cord compromise can be caused by many pathologic conditions. In this case report, a patient with Hajdu-Cheney syndrome, an exceedingly rare disorder of bony elements which led to basilar skull invagination and subsequent cervical cord compression, is described. Postoperatively, the patient continued to have difficulty with self-care and ambulation and required an extensive rehabilitation program which included mat activities, progressive resistive exercises, activities of daily living, and gait training. She was able to become independent in transfers, ambulation, and activities of daily living. The patient's rehabilitation course after cervical spinal cord decompression is presented, along with a brief description of the clinical features of this unusual disease process.