STUDY DESIGN: Retrospective case series. OBJECTIVES: To describe the indications, operative course, and outcomes in eight pediatric patients who underwent occiput-to-pelvis (O-P) spinal arthrodesis. SUMMARY: Spinal arthrodesis from the occiput to pelvis is needed for some children with extensive spine deformity. There are few reports of patients with O-P arthrodesis. METHODS: We reviewed records of pediatric patients who underwent spinal arthrodesis to treat spine deformity between 1987 and 2017 at one institution. Eight patients (six girls) who underwent staged O-P arthrodesis were identified. Underlying conditions, indications for surgery, medical comorbidities, operative courses, complications, and imaging of these patients were recorded. RESULTS: Diagnoses were neuromuscular disorders in five patients and syndromic disorders in three patients. Mean ages were 9.7 ± 4.1 years at index surgery and 16.8 ± 4.6 years at completion of O-P arthrodesis. Patients underwent a mean of three operations (range, two to five). Occipitocervical arthrodesis was the final operation in six of eight patients (all but the two patients with Loeys-Dietz syndrome). Mean follow-up after the last procedure was 8.5 ± 7.1 years. Two patients underwent revision for protruding occipital implants, and one patient underwent revision for thoracic pseudarthrosis. No patients developed postoperative infections or new neurologic deficits. At final follow-up, Scoliosis Research Society-22r questionnaire scores were lowest for Function (2.6 ± 1.0 of 5 possible points) and highest for Satisfaction (4.1 ± 1.4). CONCLUSIONS: O-P arthrodesis can benefit patients with extensive spine deformity from neuromuscular or syndromic causes. Patients were young at first operation, which may suggest that younger patients are at higher risk than older patients of major progression of deformity in other spinal regions after deformity correction. Health-related quality of life and radiographic outcomes suggest that patients who underwent O-P arthrodesis had satisfactory outcomes and maintenance of correction during a mean of 8.5 years of follow-up. LEVEL OF EVIDENCE: Level IV, therapeutic study.