Intravenous leiomyomatosis is a rare neoplastic condition characterised by nodular masses of histologically benign smooth muscle growing within veins. In most cases, the disease is confined to the pelvic veins, but involvement may extend to the inferior vena cava and right heart chamber. We report the computed tomography features of a woman who developed extensive intravenous leiomyomatosis and lung metastases, and presented with acute-onset lower limb swelling.