The incidence of nephrotic syndrome co-existing with chronic lymphocytic leukemia (CLL) is a rare condition. Almost any glomerular pathology may accompany CLL. The most frequent of all is membranoproliferative glomerulonephritis (MPGN). Moreover, in 5 - 10% of patients with CLL, monoclonal gammopathy may be detected in serum and/or urine samples. There are no well-established treatment protocols for those CLL patients with accompanying nephrotic syndrome. In this case report, we present a 55-year-old female patient diagnosed with CLL, developing nephrotic syndrome, renal dysfunction and IgG κ-type monoclonal gammopathy in the follow-up. The renal biopsy revealed glomerular and tubular deposits of κ-chain and histopathology of membranoproliferative glomerulonephritis. Rituximab along with CVP (cyclophosphamide - vincristine - prednisolone) chemotherapy regimen was initiated. At the end of 6 courses of treatment, the patient was on "nephrologic" partial remission as the serum creatinine and albumin levels had returned to normal and proteinuria decreased by more than 50%. The patient was also in partial remission for CLL. In conclusion, in patients with CLL and nephrotic syndrome, presence of MPGN along with light-chain nephropathy is rarely reported. Several different treatment protocols are discussed for these patients. Among these regimes, R-CVP is an acceptable alternative for CLL patients with MPGN. © 2008 Dustri-Verlag Dr. K. Feistle.