Background: Immunoglobulin D (IgD) myeloma is a rare subtype often described as aggressive with advanced disease at diagnosis. Primary renal involvement is seen in scarce cases.

Methods: This case features a 55-year-old man with IgD lambda myeloma presenting severe renal failure at diagnosis. Examination revealed a 10-year-old sternal plasmacytoma and multiple others in the ribs. Despite benefiting from traditional chemotherapy, he remained dependent on hemodialysis

Conclusion: Through this case, unique in the literature, we conclude that plasma cells secreting IgD can remain inactive for a long time in the form of a solitary plasmacytoma. However, in the event of medullary involvement, they can induce a myeloma with serious organic lesions.