Intraoperative neuromonitoring (IONM) is used to help detect the potential for neural injury during surgery and anesthesia. Neuromonitoring is challenging in patients with skeletal dysplasia (SD) and requires collaboration between the surgeon, anesthesiologist, and neurophysiologist. Factors such as central and peripheral neurological compromise, anesthetic agents, monitoring modalities, and expertise of the personnel involved all impact patient care. Caring for the SD population requires awareness of the individual patient disease and existing neurologic compromise.