摘要Objective: To explore clinical and laboratory features and significance of detecting serum carbohydrate antigen 125 (CA125) in immunoglobulin E (IgE) multiple myeloma. Methods: We reported the clinical findings of a male patient with IgE myeloma and elevated level of serum CA125 and reviewed the literature. Results: Laboratory tests of this patient on admission showed extremely high serum IgE and CA125, a bone marrow aspirate revealed abnormal plasma cells (38.4% of nucleated cells: 16.4% mature and 22% atypical), and in bone marrow biopsy, immunoperoxidase staining showed positive cytoplasmic staining for IgE and κ light chain within the vast majority of plasma cells. Computed tomography (CT) bone scans indicated wedge shape change and compressive fracture of thoracic vertebrae, and emission computed tomography (ECT) discovered multiple punctiform aggregation of radiation in both cervical ribs and spine. The serum IgE and CA125 gradually decreased to normal limits after eight cycles of chemotherapy. This patient is alive well with an 18-month complete remission. Conclusion: We reported the first case of IgE myeloma with elevated level of serum CA125. To further evaluate clinical characteristics and significance of CA125 in IgE myeloma, more cases are needed.