Immunoglobulin D (IgD) multiple myeloma is a rare disorder with a poor prognosis. We herein report a case of IgD myeloma presenting with renal failure and bicytopenia. A bone marrow biopsy specimen showed infiltration of homogeneous plasma cells, which stained positively for IgD and light chains. We review the literature and discuss the clinical manifestations, diagnosis and treatment strategy for this rare tumor.