Venous thrombosis is a common and severe complication in patients with cancer. We reviewed studies assessing whether a state of acquired or congenital thrombophilia influenced the risk of thrombosis in patients with cancer. The results are equivocal. However, the majority of studies were of limited size. The influence of thrombophilia in patients with cancer may be more difficult to demonstrate than in the general population, the risk of thrombosis due to cancer per se possibly outweighing the contribution of thrombophilic factors. Moreover, the results may depend on the genetic background of the population, the type of cancer, the type of thrombosis, and the chemotherapeutic treatment. Nevertheless, it appears that factor V Leiden or G20210A prothrombin gene mutation increases the risk of venous thromboembolism about 2- to 4-fold, compared with patients with cancer without either of these mutations. Similar results were observed for the occurrence of central venous catheter-associated thrombosis. Antiphospholipid antibodies and acquired resistance to activated protein C were frequently observed in patients with cancer and appeared to favor the occurrence of thrombosis. The role of hyperhomocysteinemia deserves further investigation. Since the clinical implications of these findings remain to be clarified, routine screening of cancer patients for thrombophilia cannot yet be recommended on the basis of these studies. Studies designed to assess the value of thromboprophylaxis in high-risk patients, including thrombophilic patients, with long-term central venous catheters may be valuable.