Subacute bacterial endocarditis involves microbial infection of the endocardium, heart valves, or intravascular device. Many pathogens are incriminated. Added to the clinical picture attributed to direct infection, the disease is capable of displaying a constellation of immunological and rheumatological manifestations. The diagnostic work-up includes microbiological and imaging techniques. The main rheumatological manifestations include musculoskeletal, renal, and vasculitic presentations. ANCA positivity has been reported repeatedly. We conducted a PubMed search on ANCA positivity with SBE, the results of which are discussed in this section. Attention to the possibility of SBE diagnosis in high-risk patients will lead to early diagnosis, timely treatment, and avoidance of unnecessary or potentially harmful treatment.