Our objective was to review surgical treatment options and outcomes for cervical kyphosis in patients with Larsen syndrome. Cervical kyphosis in patients with Larsen syndrome may lead to paralysis or death from spinal cord impingement at the apex of kyphosis. Proper screening and early diagnosis followed by operative stabilization have been recommended. We reviewed the literature for surgical treatment of cervical kyphotic deformity in patients with Larsen syndrome. We retrospectively reviewed medical records and used illustrative case examples to demonstrate the scenarios and treatments. Over the past 23 years, we have surgically treated many patients with Larsen syndrome who had cervical kyphosis. We have several patients who were treated initially elsewhere with posterior spinal fusion, but who required revisions for cervical kyphoses because of progression of the deformity or spinal cord impingement. This report discusses 3 illustrative patients presenting with cervical kyphoses associated with Larsen syndrome, who were initially treated with posterior spinal fusions at a young age, but required an anterior or posterior spinal fusion as a second procedure to correct non-improving progression of the deformities and spinal cord impingement. Cervical kyphosis in Larsen syndrome is a rare condition that can progress to cervical stenosis and spinal cord impingement, which may lead to paralysis or death. Several patients who were treated initially with posterior spinal fusion only required revision surgeries.