STUDY DESIGN: A retrospective study OBJECTIVE: To evaluate the efficacy and characteristics of monitoring data and outcomes during scoliosis surgery in patients with spinal muscular atrophy from Mainland China. METHOD: We analyzed somatosensory evoked potential (SSEP), motor evoked potential (MEP) records of 27 consecutive patients with spinal muscular atrophy and 59 patients with adolescent idiopathic scoliosis (AIS), who underwent spinal deformity surgery in our scoliosis center from July 2019 through August 2022. RESULTS: SSEP were elicitable in all SMA and AIS patients. The values of latencies prolonged and the amplitudes were significantly lower in SMA patients. MEP monitoring is similar in abductor pollicis brevis (APB) muscles in upper extremities of both SMA and AIS patients and combined recording from the adductor (ADD), quadriceps (QUA), tibialis anterior (TA), abductor hallucis (AH) muscles in lower extremities was necessary and effective although the elicitation rate and amplitudes in SMA patients was lower than those in AIS patients. CONCLUSION: Routine multimodality intraoperative neurophysiological monitoring (IONM) of spinal cord sensory and motor function was feasible and safe during surgical correction of scoliosis in patients with SMA, particularly when MEPs were recorded from the AH muscle combined with ADD, QUA and TA muscles.