Resistance to steroids and immunosuppression in pediatric nephrotic syndrome may be related to focal segmental glomeruloslerosis (FSGS). Rituximab, monoclonal anti-B-CD20-cell antibody is currently regarded as novel effective drug in selected cases. We describe the case of 8-years-old male pediatric patient, resistant to combined immunosuppression and presenting renal insufficiency (GFR 32.8 ml/min/1.73 m2). Patient was given overall 5 doses of rituximab [375 mg/m2/dose]. Nevertheless significant decrease of proteinuria, the further progress of renal disease was unaffected and patient developed end-stage renal failure. The efficacy of rituximab in nephrotic syndrome must be verified in controlled trials. Late onset of therapy in course of renal insufficiency might be the one of the reasons of treatment failure in our case.