**Abstract**

*Tropheryma whipplei* mainly known as the causative agent of classical Whipple's disease, also produces acute, sub-acute and chronic localized forms of infection such as endocarditis. The development of molecular tools has allowed increasing the number of cases of endocarditis due to blood culture use to be negative in *T. whipplei* endocarditis and most of the cases are confirmed post-surgery when molecular analyses of heart valves are performed. Although, *T. whipplei* endocarditis is an uncommon condition with an atypical presentation it must be considered in the diagnosis of blood culture negative endocarditis and in patients with heart failure in which valve affectation is present. Other clinical features such as long lasting arthralgia can be present in a high percentage of the patients. It is important to know that few cases are diagnosed in the context of the classical Whipple's disease. The prognosis is very good when an appropriate surgical management and antimicrobial-specific treatment is given. This chapter describes the epidemiological, clinical characteristics, diagnosis and treatments for *T. whipplei* endocarditis.

**Keywords:** *Tropheryma whipplei*, *Tropheryma whipplei* endocarditis, endocarditis, blood culture negative endocarditis, infectious endocarditis
