Non-bacterial thrombotic endocarditis (NBTE) is a disease characterised by the presence of vegetations on cardiac valves, which consist of fibrin and platelet aggregates and devoid of inflammation or bacteria. NBTE has increasingly been recognised as a condition associated with numerous diseases and a potentially life-threatening source of thromboembolism. NBTE is not a common entity; however it is frequently underestimated, probably due to underlying diseases (cancer, autoimmune disorders, HIV). NBTE is difficult to diagnose and relies on strong clinical suspicion. NBTE is also difficult to manage and each case should be individually managed by identifying and treating the underlying pathology. Surgical intervention is not recommended unless the patient is in acute congestive failure.