T. whipplei, the causative agent of classic Whipple´s disease, also produces acute, sub-acute and chronic localized forms of infection such as endocarditis. Blood culture of patients with T. whipplei endocarditis used to be negative and most of the cases are confirmed when molecular analyses of the heart valves, surgically obtained, are performed. The development of molecular tools has allowed increasing the number of cases of endocarditis due to this microorganism. This review describes the epidemiological and clinical characteristics, diagnosis, and treatments used in the 84 cases of T. whipplei endocarditis, to our knowledge, reported on the medical literature.