Central venous catheter (CVC)-related thrombosis is a frequently occurring complication and may cause significant morbidity in patients with cancer. The aim of this review is to discuss the main studies that examined whether a state of thrombophilia increases the risk of CVC-related thrombosis in patients with cancer. The studies were retrieved by an extensive Medline search. Patients with cancer with a CVC and a factor V Leiden mutation have a higher risk of developing CVC-related thrombosis than patients with cancer having a CVC without the mutation. The scarce information available suggests hyperhomocysteinemia to be a risk factor for CVC-related thrombosis. For other congenital thrombophilia factors, the available data are too limited to allow for any definitive conclusions to be made. Because the clinical implications of all these findings remain to be clarified, routine screening of patients with cancer having a CVC for thrombophilia cannot yet be recommended on the basis of the studies discussed.