Patients with skeletal dysplasia (SD) require correction, decompression, and fusion of their spine due to an extremely high incidence of kyphoscoliosis. Cervical spine pathology includes spinal canal stenosis, instability, hypoplasia, and abnormal locations of os odontoideum. Procedures to correct cervical spine abnormalities include decompression of foramen magnum and fusion of C1–C2 or other levels of the cervical spine. Cervical spine procedures may be necessary earlier in life, often under the age of 5 years, and procedures of the thoracic and lumbar spine may occur in later years Anesthetic management of the cervical or thoracolumbar spine in skeletal dysplasia patients’ needs to consider co-morbid conditions and a safe emergence in the operating room. Currently, anesthetic agents most commonly and consistently used during spine procedures are propofol, remifentanil, ketamine, and dexmedetomidine. Neuromonitoring, used to evaluate and identify potential spinal cord compromise during spine procedures, is now regarded as a standard care practice. The selection of anesthetic agents is dictated by the requirement for neuromonitoring. Upon the completion of neuromonitoring, inhalational agents such as sevoflurane are administered to maintain anesthesia while discontinuing intravenous agents such as propofol and ketamine. The primary objective to ensure a safe and awake extubation in the operating room necessitates careful adjustment of anesthetic agents. Patients are often discharged from the post-anesthesia care unit to a standard hospital unit or ward. Postoperative pain management prioritizes the use of non-steroidal anti-inflammatory drugs, such as acetaminophen and ketorolac. Additionally, non-opioid analgesics such as nalbuphine and buprenorphine are frequently employed. While there are other drugs available in this category, the aforementioned are some of the most commonly used.