Initially described in 1936, non-bacterial thrombotic endocarditis (NBTE) is a rare entity involving sterile vegetations on cardiac valves. These vegetations are usually small and friable, typically associated with hypercoagulable states of malignancy and inflammatory diseases such as systemic lupus erythematosus. Diagnosis remains challenging and is commonly made post-mortem although standard clinical methods such as echocardiography (transthoracic and transesophageal) and magnetic resonance imaging may yield the clinical diagnosis. Prognosis of NBTE is poor with very high morbidity and mortality usually related to the serious underlying conditions and high rates of systemic embolization. Therapeutic anticoagulation with unfractionated heparin has been described as useful for short term prevention of recurrent embolic events in patients with NBTE but there are no guidelines for management of this disease.