Diastematomyelia is an uncommon congenital malformation of the vertebral axis characterized by a localized longitudinal separation of the spinal cord with an interposed septum. It may be found in association with congenital scoliosis. The Authors experienced a case of congenital scoliosis with diastematomyelia. Diastematomyelia was surgicallv resected and scoliosis was corrected by Dwyer operation 5 months later. The results of both operations were satisfactory in 10 months follow up.