TAFRO syndrome is a systemic inflammatory disorder. TAFRO is an acronym that stands for thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, lymphadenopathy and hepatosplenomegaly. There are no reports of TAFRO syndrome describing cholangitis on liver biopsy. Herein, we report the first case of TAFRO syndrome with cholangitis. The patient was a 56-year-old man who presented with sudden onset abdominal pain and fever. His symptoms progressed to generalized edema, thrombocytopenia, hepatomegaly, and acute renal failure. Biopsies taken from the mediastinal lymph nodes and bone marrow showed the mixed type of multicentric Castleman's disease and mild reticulin fibrosis, respectively, compatible with TAFRO syndrome. His symptoms were temporarily relieved by steroid pulse therapy and tocilizumab. Fever and anasarca relapsed in a few weeks, however. He was then administered rituximab which resolved his symptoms almost completely.