Case report.To report the clinical and imaging findings of a patient with lumbar stenosis 44 years after posterior spinal fusion for congenital lumbar kyphosis.To our knowledge, there are no long-term follow-up reports after posterior spine fusion (PSF) for congenital kyphosis. Congenital kyphosis is an uncommon deformity with the potential to progress rapidly and result in deformity and neurologic deficits.We report the patient's history, physical examination, imaging findings, and management in addition to providing a literature review.A 54-year-old-male who underwent T8–L3 PSF in 1972 because of congenital kyphosis presented 44 years after surgery with lower back pain, buttock, and bilateral posterior leg pain. On physical examination, no weakness was elicited and magnetic resonance imaging demonstrated L4–L5 lumbar stenosis. The patient was enrolled in physical therapy and responded well to medical/interventional management.To our knowledge, this is the longest follow-up of surgical management of congenital lumbar kyphosis. Posterior fusion only halted the progression of the kyphosis with subsequent developed of adjacent segment disease distal to the fusion.Level IV.