Patients with myelomeningocele associated with severe kyphoscoliosis usually presented with rigid and angulated gibbus at their back. The condition causes this group of patients to face difficulties in their daily activities, especially in sitting and lying in supine positions. They are also prone to have a pressure sore over the gibbus and encounter the risk of infection. Here the authors would present a case of a four-year-old girl with underlying myelomeningocele who was diagnosed with worsening kyphoscoliosis along her growth. Her whole spine x-ray radiograph revealed a kyphosis angle of 80° between the T11 and L4 levels. The patient underwent a deformity corrective surgery with total kyphectomy in a combination of anterior and posterior spinal instrumentation. In the present case, we were able to obtain sufficient correction of the spinal kyphotic deformity in that patient in a single-stage surgery with satisfactory surgical outcomes at a four years follow-up.