We present the case of a 23-year-old man with steroid-resistant nephrotic syndrome due to minimal change disease who was treated with rituximab. The patient was resistant to conventional therapy. We therefore treated him with a single dose of rituximab (375 mg/m(2)). One month after the administration of rituximab, complete remission was achieved. However, six months later, the patient was administered a second dose of rituximab as the peripheral B cell counts began to recover. Thereafter, at present, that is, one year after the first rituximab administration, complete remission has been maintained. We conclude that rituximab may be an effective treatment agent for resistant nephrotic syndrome and the peripheral B cell count may be a useful marker in such patients for preventing disease relapse.