A forty-eight-year-old Japanese woman with leiomyosarcoma of the inferior vena cava presenting as Budd-Chiari syndrome is reported. A large tumor orig inating from the venous wall grew into the lumen, obstructing the hepatic vein, and extended up to the right atrium. Although this is a very rare tumor, care should be taken in order to make an early diagnosis and manage the condition effectively.
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