Background: Cases of primary malignant lymphomas of the bone are rare and account for about <1% of all lymphomas and 5% of extranodal non-Hodgkin’s lymphomas. Furthermore, most reports have described the occurrence of this disease in the middle-aged population, pediatric malignant lymphomas originating in the bone, particularly in the spine is rare. Methods: A 10-year-old boy presented with low back pain caused by T12 vertebral compression fracture due to sustaining a fall. A month later, he still presented with prolonged low back pain that intensified after exercise. A neoplastic lesion in T12 vertebral body was identified after spine computed tomography (CT) and magnetic resonance imaging. Results: We performed CT-guided biopsy, and he was diagnosed with primary malignant lymphoma of the vertebral body. He was treated with multiagent chemotherapy without irradiation, and complete remission was maintained at the 5-year follow-up. Moreover, the height of the deformed vertebral body improved as he grew. Conclusions: Herein, we report a rare case of pediatric primary malignant lymphoma of the spine with successful clinical and radiological outcome.