Simple SummaryA significant number of children surviving intra- or juxta-spinal tumors develop secondary spinal deformities and disabilities. This retrospective non-comparative study focuses on deformity analysis, age, and skeletal maturity dependent treatment options and results. Patients who developed severe scoliosis, pathological kyphosis, and/or lordosis were either treated conservatively or surgically by using growth-friendly spinal implants in younger children or definite spinal fusion during puberty. Despite severe spinal deformity, some patients were not surgically corrected in order to preserve mobility through trunk motion or malignant tumor progression. Growth-friendly spinal implants and spinal fusions were able to significantly reduce pathological curves. The first method, with bilateral rib-to-pelvis fixation, still allows spinal magnetic resonance imaging or neurosurgical intervention if needed. Severe ad hoc curve correction enhances the risk of neurological deterioration because of prior spinal cord impairment. The data of this study may help to improve the individual patient care.Childhood tumors of the central nervous system (CNS) and other entities affecting the spine are rare. Treatment options vary from surgical biopsy to partial, subtotal, and total resection, to radiation, to chemotherapy. The aim of this study is to investigate spinal deformity and subsequent surgical interventions in this patient cohort. A retrospective review at our institution identified children with CNS tumors, spinal tumors, and juxta-spinal tumors, as well as spinal deformities. Tumor entity, treatment, mobilization, and radiographic images were analyzed relative to the spinal deformity, using curve angles in two planes. Conservative or surgical interventions such as orthotic braces, growth-friendly spinal implants, and spinal fusions were evaluated and analyzed with respect to treatment results. Tumor entities in the 76 patients of this study included CNS tumors (n = 41), neurofibromatosis with spinal or paraspinal tumors (n = 14), bone tumors (n = 12), embryonal tumors (n = 7), and others (n = 2). The initial treatment consisted of surgical biopsy (n = 5), partial, subtotal, or total surgical resection (n = 59), or none (n = 12), followed by chemotherapy, radiotherapy, or both (n = 40). Out of 65 evaluated patients, 25 revealed a moderate or severe scoliotic deformity of 71° (range 21–116°), pathological thoracic kyphosis of 66° (range 50–130°), and lordosis of 61° (range 41–97°). Surgical treatment was performed on 21 patients with implantation of growth-friendly spinal implants (n = 9) as well as twelve dorsal spinal fusions (two with prior halo distraction). Surgical interventions significantly improved spinal deformities without additional neurological impairment. With the increasing number of children surviving rare tumors, attention should be focused on long-term problems such as spinal deformities and consequent disabilities. A significant number of children with CNS tumors, spinal tumors or juxta-spinal tumors required surgical intervention. Early information about spinal deformities and a close follow-up are mandatory for this patient group.