The association between venous thrombosis and malignancy, having typical features of a paraneoplastic syndrome, has been established for a century. Currently, it is recognized that arterial thromboembolism (ATE) may also behave as a paraneoplastic syndrome. Recent matched cohort studies, systematic reviews, and observational studies concur in showing an increased incidence of acute coronary events, ischemic stroke, accelerated peripheral arterial disease, and in-stent thrombosis during the 6-month period before cancer diagnosis, peaking for 30 days immediately before cancer diagnosis. Cancer patients with ATE are at higher risk of in-hospital and long-term mortality as compared with noncancer patients. In the present review, we focus on the epidemiology, clinical variants and presentation, morbidity, mortality, primary and secondary prevention, and treatment of cancer-associated ATE. The awareness that cancer can be a risk factor for ATE and that cancer therapy can initiate cardiovascular complications make it mandatory to identify high-risk patients, modify preexistent cardiovascular risk factors, and adopt effective antithrombotic prophylaxis. For ATE prophylaxis, modifiable patient-related risk factors and oncology treatment–related factors are levers for intervention. Statins and platelet antiaggregants have been studied, but their efficacy for prevention of cancer-associated ATE remains to be demonstrated. Results of revascularization procedures for cancer-associated ATE are worse than for ATE in noncancer patients. It is important that a multidisciplinary approach is adopted for making informed decisions, by involving the vascular surgeon, interventional radiologist, oncologist, and palliative medicine, as well as the patients and their family.