A 59-year-old Japanese man was admitted to our hospital with leg edema, fatigue and fever. He had a past medical history of duodenal ulcer, fatty liver, hyperuricemia and positive rheumatoid factor, but was not receiving any medication. His son had died 2 years prior to this admission after following a clinical course that suggested TAFRO syndrome.The patient showed mild fever (about 37-38 °C), polypnea and severe leg edema on admission. Chest examination revealed decreased breath sounds in the right lung. No superficial lymph nodes were palpable. Laboratory tests on admission revealed mild thrombocytopenia, hypoproteinemia, elevation of C-reactive protein (CRP), alkaline phosphatase (ALP), creatinine (Cr), and urine N-acetyl-β-d-glucosaminidase (NAG) ( Table 1). Urine testing showed granular casts without proteinuria or hematuria. Concentrations of aspartate aminotransferase (AST) and alanine transaminase (ALT) were elevated, but unchanged compared with the results of a medical check 6 months before admission, and so were attributed to fatty liver. Chest X-ray and computed tomography (CT) showed mild lymphadenopathy in the mediastinum, bilateral pleural effusion and ascites (Fig. 1).Blood, sputum and urine culture tests were all negative and no other infectious diseases were present. Negative results were obtained for all autoantibodies. Among the tumor markers, only soluble interleukin-2 receptor (sIL-2R) was elevated. Bone marrow biopsy was performed to rule out malignant lymphoma, revealing normocellular Abstract Successful use of tocilizumab (TCZ) to treat TAFRO syndrome has recently been reported. In those cases, TCZ was used with steroid. We present herein the case of a 59-year-old man with very severe TAFRO syndrome who was successfully treated using TCZ without steroid. He showed rapidly progressive anasarca, acute renal failure and very severe thrombocytopenia. We initially used steroid, but its efficacy was limited. Moreover, steroid use had to be stopped as soon as possible, because hemorrhagic shock developed due to severe duodenal ulcer. After overcoming infections (about 40 days after stopping steroid), administration of TCZ was started and the patient was discharged in clinical remission.