Primary extra nodal lymphoma of bone means the malignant lymphoma presents initially in bones. It is a rare disease of unknown etiology in children. The disorder mainly manifests in localized bone lesions. Bone arthrodynia and localized bone swelling are the most common symptoms, with or without systemic symptoms. Radiologic examinations reveal osteolytic lesions. The histological features are various, and the majority of histology are diffuse large B cell lymphomas. Diagnosis of the disease may be confirmed by bone biopsy. The prognosis of the patients with this disorder is comparatively favorable after general treatment with chemotherapy and radiation therapy.