Purpose Early onset spinal deformities (EOSD) can be life-threatening in very young children. In the growing spine, surgical intervention is often unavoidable and should be carried out as soon as possible. A deformed section of the spine not only affects the development of the remaining healthy spine, but also that of the chest wall (which influences pulmonary function), the extremities and body balance. Posterior vertebral column resection (PVCR) represents an effective surgical solution to address such problems. However, reports in the literature concerning PVCR are mostly limited to its use in adolescents or adults. The purpose of this study was to illustrate our experience with PVCR in EOSD and to describe the surgical technique with respect to the unique anatomy of young children. Materials and methods Four children [mean age 3.7 (range 2.5-5.2) years] with severe spinal deformity underwent PVCR through a single approach. Multimodal intraoperative monitoring was used in all cases. Surgery included one stage posterior circumferential resection of one vertebral body along with the adjoining intervertebral discs and removal of all posterior elements. A transpedicular screw-rod system was used for correction and stabilisation. Fusion was strictly limited to the resection site, allowing for later conversion into a growing rod construct at the remaining spine, if necessary. Relevant data were extracted retrospectively from patient charts and long spine radiographs. Results The mean operation time was 500 (range 463-541) min, with an estimated blood loss of 762 (range 600-1,050) ml. Mean follow-up time was 6.3 (range 3.5-12.4) years. After PVCR, the mean Cobb angle for scoliosis was reduced from 69°(range 50-99°) to 29°(5-44°) and the sagittal curvature (kyphosis) from 126°(87-151°) to 61°(47-75°). The mean correction of scoliosis was 57 % (18-92°) and of kyphosis, 51 % (44-62°). There were no spinal cord-related complications. In three patients, spinal instrumentation for growth guidance (fusion less growing rod technique) was applied. Two patients had complications: one patient had a complication of anesthesia, halo pin failure, and revision surgery with extension of the instrumentation cranially due to loss of correction; the second patient had a postoperative infection, which required plastic reconstructive measures. Conclusion PVCR appears to be an effective technique to treat severe EOSD. There are important differences in its use in young children when compared with older patients. In patients with EOSD, additional surgical procedures are often necessary during growth, and hence non-fusion instrumentation beyond the vertebral resection site is advantageous, as it permits spinal growth and the later addition of fusion.