Diamond Blackfan anemia (DBA) is a rare disease characterized by aplasia or hypoplasia of erythroid lineage. Normochromic, usually macrocytic. but occasionally normocytic anemia and reticulocytopenia are characteristic findings of DBA. DBA is associated with an increased risk of malignancy. Most of the reported malignancies are acute myeloid leukemia. Solid tumors including hepatocellular carcinoma and osteosarcoma have also been identified. We could find 29 reported cases with DBA and malignancy. Two of them were diagnosed as Hodgkin lymphoma at 15 and 23 years. respectively. Here we report a 7-year-old boy with DBA who developed Hodgkin disease.