Congenital malformation complex of the spine and the spinal cord can be a syndromic entity rather than a symptom complex. The spinal cord lesion is usually bilaterally symmetrical, but, there are occasional cases with one or more hemivertebrae, often associated with a central bony spur splitting the cord (diastematomyelia), in which one leg is virtually normal while the other is severely paralysed. Hemimyelomeningocele over the lumbar area may be associated with extensive spine malsegmentation compatible with the diagnosis of spondylocostal dysplasia syndrome. In this report, we present a 3-year-old girl who underwent neurological evaluation and spinal imaging studies for extensive spine malsegmentation compatible with spondylocostal dysostosis syndrome associated with hemimyelomeningocele. She had a series of corrective orthopaedic interventions to reconstruct her pelvic girdle and spine deformities, with a satisfactory outcome.