Background: There have been many reports on the treatment of congenital kyphoscoliosis. However, congenital deformities in the cervicothoracic spine (C7-T1) have not been well described because of the rarity of these conditions. Methods: The medical records and imaging studies of 25 children who were treated with 360 degrees osteotomy for congenital deformities in the cervicothoracic spine (C7-T1) at a mean age of 11.4 years were reviewed. Results: All 25 children presented with torticollis; 4 presented with neck pain; 10, with facial asymmetry; and 3, with preoperative neurological deficits. Twenty-three patients had congenital deformities in other regions of the spine. Six patients had a total of 8 intraspinal deformities. On average, the cervicothoracic curve was corrected from 53 degrees preoperatively to 14 degrees at the latest follow-up, the segmental kyphosis was corrected from 25 degrees to 12 degrees, and the head tilt improved from 25 degrees to 5 degrees. Nineteen patients had a total of 28 complications, including 1 transient cord injury together with a permanent C8 nerve root injury, 11 transient nerve root injuries, 1 transient Homer syndrome, 9 cases of decompensation of a compensatory curve, 2 implant failures, 2 cases of hemothorax, 1 dural tear, and 1 case of delayed wound-healing. Conclusions: Most congenital cervicothoracic deformities are fixed, and early surgical intervention may be needed. A 360 degrees osteotomy is indicated for this type of rigid deformity and may provide satisfactory correction. However, 360 degrees osteotomy in the cervicothoracic spine (C7-T1) is technically demanding with a higher risk of nerve root injuries, although most injuries tend to be transient. If the compensatory thoracic curve is severe and rigid, 1-stage or staged surgery in this region may be required.