BACKGROUND Multiple calcified primary central nervous system lymphoma (PCNSL) is extremely rare in childhood. METHODS We report a 4-year-old boy suffering from multiple calcified B-cell lymphoma in the brain with immunodeficiency. RESULTS The boy had a history of walking weakness and seizure for 4 months. The serum levels of immunoglobulin G, A and M were decreased. Brain MRI showed multiple lesions which had ring enhancement. CT showed calcification in all of the lesions. The boy was firstly misdiagnosed with multiple chronic brain abscesses. Pathological analysis of biopsy confirmed the diagnosis of anaplastic diffuse large B-cell lymphoma. CONCLUSION PCNSL should be included in the differential diagnosis of intracranial mass with calcification.