We describe a patient with a primary renal plasmacytoma. In the litera
ture available, only six clinical cases have been reported. A 64-year-
old patient is presented who had the clinical signs of a renal cell ca
rcinoma. Histological examination after nephrectomy, however, revealed
a plasmacytoma. Based on this case, we discuss how one should proceed
in this disease. Primary renal plasmacytoma can be tentatively diagno
sed preoperatively only in the presence of paraproteinemia or Bence-Jo
nes proteinuria, as it cannot be distinguished from other renal tumors
by imaging procedures. Postoperatively, further staging procedures mu
st rule out bone involvement (solitary myeloma or multiple myeloma). N
ephrectomy is required in patients with plasmacytoma only if renal com
plications occur.