A patient who was treated with hemodialysis in a foreign country was unexpectedly found to have Renal limited vasculitis (RLV) by renal biopsy and was successfully treated in time. As the diagnosis and treatment process was very enlightening, it is reported as follows. The patient was a female, 48 years old, from Jiangxi. She was admitted to the hospital because she was “found to have generalized swelling for half a year and had hemodialysis for 2 months”. In October 2006, the patient was found to have swelling of face and both lower extremities with increased nocturia. In January 07, he went to several hospitals in Shanghai, but to no avail. At that time, his blood creatinine rose to 400umol/l. One month later, his blood creatinine rose to 1000umol/l. He returned to the local hospital and started maintenance hemodialysis. However, due to the poor vascular condition, the A-V fistula surgery at the local hospital failed, so he came to our department again for A-V fistula reconstruction. After the successful A-V endovascular fistula and maintenance hemodialysis were performed to improve the symptoms, we found that the patient had a relatively short time of onset and the ultrasound indicated that the size of both kidneys was close to normal. After consulting with the patient and his family, a renal biopsy was performed under ultrasound guidance on 3.29. 4.6 The renal pathology report indicated focal segmental hyperplasia and focal segmental necrotizing glomerulonephritis with acute interstitial nephritis with localized crescent formation and no immune precipitates were seen. Although the patient’s chest X-ray and immune panel were normal, the possibility of “vasculitis” could not be ruled out, and ANCA-related indexes were given immediately. 4.19 Anti-neutrophil plasma antibody (IIF) was positive for P-ANCA1:1280, and anti-neutrophil plasma antibody target antigen MPO-ANCA. 262.48: At this time, the diagnosis of “renal restrictive vasculitis, acute glomerulonephritis” was clear, and in order to save the kidney on the verge of consolidation, methylprednisolone 500mg and CTX0.6 shock therapy were given immediately on top of maintenance hemodialysis, followed by regular prednisone 50mg,qd+CTX0.4,qm maintenance therapy. On 4.23, the patient’s blood creatinine dropped to 381umol/l, and he was successfully discharged from hemodialysis treatment and improved. Discussion Renal Restrictive Vasculitis (RLV) is a type of vasculitis that is considered by many to be an idiopathic rapidly progressive glomerulonephritis without immune precipitation, and by others to have microvascular immune damage. It is more difficult to diagnose clinically because it is unlike Wegener’s granulomatosis and microscopic vasculitis in which multiple organ damage is present. Therefore, it, like ANCA-negative vasculitis, becomes particularly important to diagnose with a renal biopsy. If diagnosed early and treated early with hormones and cyclophosphamide, the results are better, with a kidney survival rate of more than 50%.