Congenital scoliosis accounts for 10% of all scolioses which require treatment. Correct treatment demands an exact and early detection of the deformity. Early intervention may only require a minor operation. Two cases are presented with paraplegia as the first presenting symptom of congenital kyphoscoliosis. In both cases anterior decompression and spondylodesis was followed by early neurological recovery. After a second operation with posterior spondylodesis and instrumentation ad modum Cotrel-Dubousset the patients were mobilised. Patients with congenital spinal deformity ought to be investigated and treated in a highly specialized department of spinal surgery.