Central core disease is congenital myopathy, pathologically distinguished by the presence of well-demarcated round lesion or cores within muscle fibers. A 8-year-old female patient, who had severe thoracolumbar scoliosis, has done an open muscle biopsy and surgical correction of scoliosis. The muscle pathology showed central core with type 1 fiber predominance. After the surgical correction, her ability of sitting feature was improved. Surgical interventions may be considered in congenital myopathies with severe scoliosis for improving quality of life.