Background: Pediatric intraspinal space-occupying lesions are relatively uncommon. However, these lesions can result in neurological disabilities due to misdiagnosis and delayed treatment. The main goal of the present study is to evaluate the clinical and radiological features and treatment options of pediatric intraspinal space-occupying lesions in order to improve the clinical recognition and management. Methods: Clinical data of 27 children with intraspinal space-occupying lesions who underwent surgery treatment in a tertiary-care hospital between 2010 and 2018 were retrospectively reviewed and analyzed. Results: Of these 27 patients, 14 (51.85%) were girls and 13 (48.15%) were boys. The most common age group affected was 10~14 years (62.96%, 9 girls and 8 boys in this age group). The mean age was 10.11 years old. Pain and weakness were the most common clinical symptoms. Preoperative magnetic resonance imaging (MRI) identified intramedullary (10 cases, 37.04%), intradural extramedullary (10 cases, 37.04%) and extradural (7 cases, 25.92%) lesions, respectively. The majority of the lesions were intraspinal tumors (23 cases, 85.19%). The histological diagnosis of tumors included embryonic residual tumors (6 cases, 22.22%), ependymoma (5 cases, 18.52%), primitive neuroectodermal tumors (PNET) (3 cases, 11.11%), schwannomas (2 cases, 7.4%), ganglioneuroma (1 case, 3.7%), Ewing’s sarc (1 case, 3.7%), B-cell non Hodgkin lymphoma (1 case, 3.7%), Hodgkin lymphoma (1 case, 3.7%), chondrosarcoma (1 case, 3.7%), ganglioglioma (1 case, 3.7%), and glioma (1 case, 3.7%). Conclusions: The incidence of pediatric intraspinal space-occupying lesions is low, and the clinical manifestation is lack of specificity. The prognosis for children with malignant tumors is poor and surgical resection is still the primary treatment option.