In order to perform an accurate review starting with the reported case, which included two major significant pathologies, both a genetically inherited disease, the ACTA2 variant gene leading to a familial thoracic aortic aneurysm and dissection, as well as a rare infectious disease, namely blood culture-negative infective endocarditis on a mechanical aortic valve, the research on the medical literature started by looking for the coexistence of both pathologies, but it failed to identify any patient similar to the case presented above. Here, ACTA2 is linked to infectious disease.