The study is to improve clinicians’ understanding of TAFRO syndrome, to explore the diagnosis and treatment of TAFRO syndrome and to identify TAFRO syndrome in the early stage. The clinical manifestations, laboratory examination results, imaging manifestations, diagnosis and treatment of TAFRO syndrome were reported, and the literature of TAFRO syndrome was reviewed. The main clinical manifestations of the female were intermittent vaginal bleeding, fever, depressive edema of both legs, red blood cell and thrombocytopenia, and renal function deterioration. The results showed that leukocytes increased, anemia, thrombocytopenia and severe renal dysfunction were found; With fever, C-reactive protein and procalcitonin increased significantly, bone penetration suggested that granulocyte proliferation was active, and megakaryocytes were seen. But anti-infection treatment was ineffective; CT suggested that there was a high uptake of multiple fluorodeoxyglucose (FDG) in many parts of the body; The lymph node biopsy was considered to be in accordance with the transparent vascular type of Castleman disease; Renal biopsy was used to return thrombotic microvascular disease with subacute renal tubulointerstitial nephropathy. In terms of treatment, the clinical condition of the patients was improved after methylprednisolone (60 mg, once a day), the temperature was normal, and the effusion in the serous cavity was better than before. The blood transfusion and platelet support therapy were intermittently given. Hemoglobin and platelets were increased in sex, and the urine volume increased to 1 000 mL/day. However, the platelet dropped at a later time, after 1 month of treatment with topizumab, the clinical condition of the patients was further improved. At present, the blood pigment and platelets returned to normal and had been separated from dialysis. TAFRO syndrome is a special subtype of idiopathic multicentric Castleman disease, and it is a group of systemic inflammatory diseases with its own characteristics. Its clinical manifestations and diagnosis and treatment are unique compared with other idiopathic multicentric Castleman diseases. For the enlargement of lymph nodes of unknown reasons, it is suggested to improve the lymph node biopsy actively. Renal insufficiency is an important part of TAFRO syndrome. Renal biopsy is of great significance to study the pathogenesis of TAFRO syndrome and to judge the prognosis of patients. The clinical diagnosis of the disease requires comprehensive clinical manifestations and the results of various examinations. Early diagnosis and early treatment of the disease can often achieve good clinical effect.