Ellis-Van Creveld (EVC) syndrome is one of the entities belonging to the skeletal ciliopathies. Scoliosis is a rare clinical manifestation of EVC syndrome. This article reports a case of a 14-year-old female patient who was admitted to the hospital due to “Scoliosis found for 3 years”. She has multiple skeletal abnormalities, short stature, polydactyly, single atrium, etc.at the same time. She was diagnosed with EVC syndrome by detecting a compound heterozygous mutation of EVC gene. By reviewing the relevant literature, the mechanisms of scoliosis were mainly explained by the involvement of EVC genes in the Hh and Wnt/β-catenin signaling pathways. Premature mineralization of the pedicle cause by relative anterior spinal overgrowth, the mechanical properties of the spine is decreased cause by the disorder of spinal mineralization, abnormal development of intervertebral disc and muscle.These mechanisms may change the biomechanics of the spine and cause spinal deformity. In the orthopedic operation of scoliosis in patients with EVC syndrome,we should pay attention to the influence of common complications during the perioperative period, such as severe heart disease and thoracic stenosis.