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Intravascular large B-cell lymphoma (IVLBCL) is an extremely rare subtype of large B-cell lymphoma characterized by the presence of lymphoma cells within the lumen of small blood vessels. IVLBCL presents with nonspecific symptoms such as fever, weight loss, and bleeding. Because of its rarity and unremarkable clinical presentation, a timely diagnosis is very challenging.A 71-year-old Korean man complained of fever, but apart from pretibial pitting edema and mild thrombocytopenia, the physical examination and laboratory test findings were unremarkable.A bone marrow biopsy was also nonspecific. The fever persisted and his thrombocytopenia became more pronounced, prompting further laboratory tests that indicated infiltrative liver disease.Because of coagulopathy, a liver biopsy was performed using a transjugular instead of apercutaneous approach.The procedure was performed without complications, and the pathologic examination findings were consistent with IVLBCL. Unfortunately, the patient died because of disease progression before treatment could be administered.Given that an early diagnosis can affect the prognosis of IVLBCL, quickly and safely obtaining a biopsy specimen is very important. The case presented here shows that a liver biopsy obtained via a transjugular approach is safe and could be preferentially considered when there is a high risk for bleeding complications.