Liver cirrhosis is an increasing cause of mortality and morbidity in developed countries. Infective Endocarditis (IE) is an uncommon disease with notable morbidity and mortality. Even though cirrhosis is associated with immune dysfunction and increased occurrence of bacterial infection, IE is infrequently diagnosed in these patients. Thus, the purpose of this study was to systematically review all published cases of IE in patients with cirrhosis in the literature. A systematic review of PubMed, Scopus and Cochrane (through 23 April 2020) for studies providing epidemiological, clinical, microbiological as well as treatment data and outcomes of IE in patients with cirrhosis was performed. A total of 78 studies, containing data of 602 patients, were included. A prosthetic valve was present in 17.8%, while the most common causative pathogen was in 26% followed by spp in 16.8%. Aortic valve was the most commonly infected site, followed by mitral valve. Diagnosis was set with a transthoracic ultrasound in 55.2%, while the diagnosis was set at autopsy in 16.7%. Fever and heart failure were the most common clinical presentations. Aminoglycosides, vancomycin, and cephalosporins were the antimicrobials most frequently used for treatment. Clinical cure was noted in 68.2%, while overall mortality was 41.4%. This systematic review thoroughly describes IE in patients with liver cirrhosis and provides information on epidemiology, clinical presentation, treatment and outcomes.