Therapeutic options for the management of venous thromboembolism (VTE) in patients with cancer remain very limited. Although low-molecular-weight heparin monotherapy has been identified as a simple and efficacious regimen compared with an initial parenteral anticoagulant followed by long-term therapy with a vitamin K antagonist, many clinical questions remain unanswered. These include optimal duration of anticoagulant therapy, treatment of recurrent VTE, and the treatment of patients with concurrent bleeding or those with a high risk of bleeding. Treatment recommendations from consensus clinical guidelines are largely based on retrospective reports or extrapolated data from the noncancer population with VTE, as randomized controlled trials focused on cancer-associated thrombosis are sorely lacking. Furthermore, with improvements in imaging technology and extended survival duration of patients with cancer, we are encountering more unique challenges, such as the management of incidental VTE. Clinicians should be aware of the limitations of the novel oral anticoagulants and avoid the use of these agents because of the paucity of evidence in the treatment of cancer-associated thrombosis.