Introduction: Diffuse large B-cell lymphoma (DLBL) is one of the most common malignant lymphomas in children. Although it can arise anywhere of the body, childhood DLBL isolated to the scalp without metastasis is very uncommon. We experienced a case of isolated DLBL of the scalp in a child. Case Report: A 13-year-old, previously healthy boy visited outside hospital, because of painless enlarged mass in the scalp of occipital area from 2 months prior to visit. He had computed tomography and it revealed an about 6.2 cm-sized ovoid mass in the left occipital scalp. He underwent excisional biopsy, and microscopic finding of the tumor was consistent with malignancy, favoring lymphoma. For further evaluations and treatment, she was transferred to our hospital. In our hospital, re-evaluation of pathology was performed. In microscopic finding, the tumor consists of many homogenous small blue round cells, and staining of the tumor was positive for CD20, Bcl-2, and Ki-67, but negative for CD3, CD10, Bcl-6,. NUM-1, which was consistent with DLBL. Polymerase chain reaction for IgH chain gene rearrangement of the tumor was also positive. There was no other finding indicating residual mass, distant metastasis or marrow/CSF involvement. According to the diagnosis of group A DLBL, he received 2 courses of chemotherapy (BFM90 regimen), which consisted of dexamethasone, methotrexate, ifosfamide, cytarabine, etoposide, cyclophosphamide, doxorubicin, and intrathecal therapy. He completed chemotherapy with no definite adverse effect, and was followed at the outpatient clinic for about 1 years, without any evidence of relapse. Conclusion: The authors report a rare case of DLBL isolated to the scalp in a 13-year-old boy. Despite its rarity, DLBL should be included in the differential diagnosis of scalp tumors in children or adolescents, such as Langerhans cell histiocytosis.