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Thrombosis of the inferior vena cava is a rare clinical expression of primary antiphospholipid syndrome. This case clearly illustrates the clinical manifestations, work-up findings and management principles of thrombosis of the inferior vena cava in primary antiphospholipid syndrome before the stage of complication. The patient was a 24-year-old female with a history of recurrent deep venous thrombosis. She was admitted to the department of internal medicine for dyspnea and pain of the right hypochondria. Physical examination disclosed an edematous ascitic syndrome. The scanner coupled with Doppler ultrasonography showed thrombosis of the inferior vena cava. On these radiological findings, an immunological work-up was requested, which showed the presence of antiphospholipid antibodies., (Copyright 2009 Elsevier Masson SAS. All rights reserved.)